This document provides an overview of diffuse parenchymal lung disease (DPLD) and idiopathic interstitial pneumonias (IIPs). It discusses the classification of IIPs including idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and others. It also covers the clinical presentation, diagnostic approach involving history, physical exam, pulmonary function tests, radiological findings on high-resolution CT, and role of bronchoscopy with bronchoalveolar lavage in evaluating these conditions. Key points like reduced diffusing capacity on pulmonary function tests and honeycombing on imaging in IPF
DLCO/TLCO measures the ability of the lungs to transfer carbon monoxide from the alveoli to the blood. It estimates the surface area and thickness of the alveolar-capillary membrane. CO is used instead of oxygen because its transfer is diffusion limited and it binds readily to hemoglobin. A single-breath hold method is most common where the patient inhales a gas mixture and holds their breath for 10 seconds while CO uptake is measured. DLCO can help identify interstitial lung diseases, emphysema, pulmonary hypertension and assess treatment response. Reduced DLCO may be due to decreased surface area from conditions like emphysema, or increased membrane thickness from fibrosis. Adjustments are made
1. The document discusses a seminar on interstitial lung disease (ILD) that will cover the definition, classification, epidemiology, diagnostic approach, and radiological and histopathological patterns of common ILDs.
2. Key topics that will be covered include the most common types of ILD in India and worldwide, HRCT and pathology patterns of common ILDs, anti-fibrotic agents, progressive fibrosing ILDs, and supportive care including vaccination, oxygen therapy, pulmonary rehabilitation, and lung transplantation.
3. The diagnostic approach involves a clinical evaluation including history, exam, pulmonary function tests, radiology with chest X-ray and HRCT, and pathology including bronchoscopy and
This document discusses methods for assessing small airway function, including spirometry, plethysmography, dynamic compliance testing, inert gas washout tests, impulse oscillometry, and exhaled nitric oxide. Spirometry measures like FEF25-75 and FEV3/FVC ratio provide information about small airways, while plethysmography evaluates gas trapping through residual volume. Dynamic compliance is reduced with uneven ventilation. Inert gas washout tests measure gas mixing efficiency through the lung clearance index. Impulse oscillometry evaluates resistance, reactance, and frequency dependence to detect central and peripheral airway obstruction.
Approach To Diffuse Parenchymal Lung DiseasesGamal Agmy
This document provides an overview of interstitial lung diseases (ILD), also known as diffuse parenchymal lung diseases (DPLD). It begins by defining the pulmonary interstitium and reviewing the spectrum of ILD. Common clinical presentations are discussed. The document then reviews approaches to diagnosis, including history, physical exam, imaging like chest x-ray and CT, pulmonary function tests, and lung sampling. Common radiographic patterns seen in ILD like ground glass, reticulation, nodules and cysts are also summarized.
An update on the management of Idiopathic Pulmonary Fibrosis (IPF)Sarfraz Saleemi
This document provides a historical overview and update on the management of idiopathic pulmonary fibrosis (IPF). It discusses the evolution of IPF diagnosis and classification over time based on clinical and pathological criteria. Key risk factors for IPF like older age, family history, smoking and genetics are summarized. The document also reviews prognostic indicators, comorbidities, current pharmacological therapies including pirfenidone and nintedanib, and the multidisciplinary approach to diagnosis and management of IPF.
This document discusses diffuse parenchymal lung diseases (DPLD), also known as interstitial lung diseases. It describes the different categories and subtypes of DPLD, including idiopathic interstitial pneumonias (IIP) such as idiopathic pulmonary fibrosis (IPF). IPF is the most important subtype of IIP, with a poor prognosis. The document outlines approaches to diagnosing and treating IPF.
Practical approach to interstitial lung diseases Hamdi Turkey
These lecture notes were prepared by Dr. Hamdi Turkey- Pulmonologist- Department of internal medicine - Taiz university
Do Not Forget To Visit Our Pages On Facebook on the following Links:
https://www.facebook.com/groups/569435236444761/
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Allergic Broncho Pulmonary Aspergillosis (ABPA) by Dr.Tinku JosephDr.Tinku Joseph
HRCT is more sensitive than CXR in detecting bronchiectasis and other pulmonary changes in ABPA. It helps establish the diagnosis and assess disease severity and response to treatment.
DLCO/TLCO measures the ability of the lungs to transfer carbon monoxide from the alveoli to the blood. It estimates the surface area and thickness of the alveolar-capillary membrane. CO is used instead of oxygen because its transfer is diffusion limited and it binds readily to hemoglobin. A single-breath hold method is most common where the patient inhales a gas mixture and holds their breath for 10 seconds while CO uptake is measured. DLCO can help identify interstitial lung diseases, emphysema, pulmonary hypertension and assess treatment response. Reduced DLCO may be due to decreased surface area from conditions like emphysema, or increased membrane thickness from fibrosis. Adjustments are made
1. The document discusses a seminar on interstitial lung disease (ILD) that will cover the definition, classification, epidemiology, diagnostic approach, and radiological and histopathological patterns of common ILDs.
2. Key topics that will be covered include the most common types of ILD in India and worldwide, HRCT and pathology patterns of common ILDs, anti-fibrotic agents, progressive fibrosing ILDs, and supportive care including vaccination, oxygen therapy, pulmonary rehabilitation, and lung transplantation.
3. The diagnostic approach involves a clinical evaluation including history, exam, pulmonary function tests, radiology with chest X-ray and HRCT, and pathology including bronchoscopy and
This document discusses methods for assessing small airway function, including spirometry, plethysmography, dynamic compliance testing, inert gas washout tests, impulse oscillometry, and exhaled nitric oxide. Spirometry measures like FEF25-75 and FEV3/FVC ratio provide information about small airways, while plethysmography evaluates gas trapping through residual volume. Dynamic compliance is reduced with uneven ventilation. Inert gas washout tests measure gas mixing efficiency through the lung clearance index. Impulse oscillometry evaluates resistance, reactance, and frequency dependence to detect central and peripheral airway obstruction.
Approach To Diffuse Parenchymal Lung DiseasesGamal Agmy
This document provides an overview of interstitial lung diseases (ILD), also known as diffuse parenchymal lung diseases (DPLD). It begins by defining the pulmonary interstitium and reviewing the spectrum of ILD. Common clinical presentations are discussed. The document then reviews approaches to diagnosis, including history, physical exam, imaging like chest x-ray and CT, pulmonary function tests, and lung sampling. Common radiographic patterns seen in ILD like ground glass, reticulation, nodules and cysts are also summarized.
An update on the management of Idiopathic Pulmonary Fibrosis (IPF)Sarfraz Saleemi
This document provides a historical overview and update on the management of idiopathic pulmonary fibrosis (IPF). It discusses the evolution of IPF diagnosis and classification over time based on clinical and pathological criteria. Key risk factors for IPF like older age, family history, smoking and genetics are summarized. The document also reviews prognostic indicators, comorbidities, current pharmacological therapies including pirfenidone and nintedanib, and the multidisciplinary approach to diagnosis and management of IPF.
This document discusses diffuse parenchymal lung diseases (DPLD), also known as interstitial lung diseases. It describes the different categories and subtypes of DPLD, including idiopathic interstitial pneumonias (IIP) such as idiopathic pulmonary fibrosis (IPF). IPF is the most important subtype of IIP, with a poor prognosis. The document outlines approaches to diagnosing and treating IPF.
Practical approach to interstitial lung diseases Hamdi Turkey
These lecture notes were prepared by Dr. Hamdi Turkey- Pulmonologist- Department of internal medicine - Taiz university
Do Not Forget To Visit Our Pages On Facebook on the following Links:
https://www.facebook.com/groups/569435236444761/
AND
https://www.facebook.com/groups/690331650977113/
Allergic Broncho Pulmonary Aspergillosis (ABPA) by Dr.Tinku JosephDr.Tinku Joseph
HRCT is more sensitive than CXR in detecting bronchiectasis and other pulmonary changes in ABPA. It helps establish the diagnosis and assess disease severity and response to treatment.
ABPA is a complex immunological lung disorder caused by hypersensitivity to Aspergillus fumigatus antigens in patients with asthma or cystic fibrosis. It presents as poorly controlled asthma, bronchiectasis, or recurrent lung infiltrates. Diagnosis requires elevated total IgE levels, positive skin test or specific IgE to A. fumigatus, and meeting two of three criteria including precipitating antibodies, chest imaging findings, or eosinophil count. Treatment involves oral steroids and long-term antifungal therapy to reduce IgE levels and control symptoms. The disease has a relapsing course and complications include worsening asthma and permanent lung damage if not treated early.
The document discusses interstitial lung disease (ILD), including its common features, types, causes, diagnostic approach and treatment. It describes various ILD types such as idiopathic pulmonary fibrosis and sarcoidosis. Imaging and biopsy are used to diagnose ILD and determine prognosis. Treatment involves identifying and removing environmental causes, suppressing inflammation, and managing complications like right heart failure.
The document discusses various COPD phenotypes including:
1) Asthma-COPD overlap phenotype characterized by incompletely reversible airway obstruction and asthma-like features.
2) Frequent exacerbator phenotype defined as 2 or more exacerbations per year which increases health risks.
3) Upper lobe-predominant emphysema phenotype where surgical lung volume reduction may help.
4) Infrequent exacerbator phenotype experiencing less than two exacerbations per year requiring only bronchodilators.
5) Alpha-1 antitrypsin deficiency phenotype which is a genetic cause of panlobular emphysema.
FlashPath - Lung - Cryptogenic Organizing Pneumonia (Bronchiolitis Obliterans...Hazem Ali
This document discusses cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia (BOOP). It presents the following key points:
1. COP is characterized by subacute onset of respiratory symptoms and radiologic evidence of patchy consolidation and nodularity. Histologically, it shows intraluminal plugs of granulation tissue (Masson bodies) in the respiratory bronchioles and alveolar ducts.
2. COP has an excellent prognosis and is usually steroid responsive. However, steroid resistance or relapse may occur after treatment is stopped.
3. The pathology of COP must be differentiated from other interstitial lung diseases that present similarly such as usual inter
Practical approach to Idiopathic Pulmonary Fibrosis.Hiba Ashibany
This document provides information on idiopathic pulmonary fibrosis (IPF), including its causes, diagnosis, clinical features, prognosis, and treatment approaches. It summarizes that IPF is a progressive lung disease of unknown cause where scarring develops in the lungs. Diagnosis involves ruling out other conditions, imaging, and sometimes biopsies. Prognosis is generally poor with median survival of 3 years. Treatment includes drugs like pirfenidone and nintedanib that can slow disease progression in mild to moderate IPF.
This document summarizes various connective tissue disease-associated interstitial lung diseases. It describes common intrathoracic manifestations and imaging findings for conditions like rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus, and polymyositis/dermatomyositis. For each condition, it lists typical radiological patterns seen on CT such as ground glass opacities, reticulation, consolidation, and honeycombing. Photomicrographs are also included to illustrate histopathological findings for some of the interstitial lung diseases.
IPF AND PROGRESSIVE PULMONARY FIBROSIS 2022UPDATE(ATS.pptxAnjanaAnilkumar14
This document provides updates on the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) from the ATS/ERS:
- For IPF, a conditional recommendation was made for transbronchial lung cryobiopsy as an alternative to surgical lung biopsy. No recommendation was given for or against genomic testing. Antacid medications and antireflux surgery are not recommended for treating IPF.
- PPF is defined as worsening symptoms, radiological progression, and physiological progression within a year in an ILD other than IPF with no alternative explanation. A conditional recommendation was made for nintedanib for PPF, and more research on pirfenidone
This document provides a summary of an presentation on approaches to interstitial lung disease (ILD) and updates in idiopathic pulmonary fibrosis (IPF) management. It begins with an introduction to ILDs and the pulmonary interstitium. It then covers the pathogenesis, classification, epidemiology, clinical assessment including history, exams, tests and tissue sampling, and radiological and pathological findings of ILDs. A significant portion discusses IPF specifically, including prognosis, guidelines for diagnosis, and medical therapies including pirfenidone and nintedanib which have been shown to reduce lung function decline in clinical trials. It concludes with experience using pirfenidone in Saudi Arabia.
Pulmonary sarcoidosis is a multisystem inflammatory disease of unknown etiology characterized by non-caseating granulomas. It most commonly affects the lungs, skin, eyes and lymph nodes. The pathogenesis involves accumulation of inflammatory cells and T lymphocytes forming granulomas that can damage tissues. Diagnosis is based on clinical features, radiological evidence of non-caseating granulomas on biopsy with other causes excluded. Treatment depends on severity and organ involvement but may include corticosteroids.
This document discusses pulmonary function tests (PFTs), including their goals, uses, limitations, procedures, and interpretations. PFTs are used to assess lung function before surgeries and characterize any pulmonary dysfunction. Key information obtained from PFTs includes measurements of forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), their ratio (FEV1/FVC), and peak expiratory flow rate. Interpretations of these values can indicate restrictive or obstructive lung disease. The document outlines how PFTs are performed using portable devices or clinic spirometers and flow-volume loops.
This document describes a case of a 48-year-old male admitted with hemoptysis and cough who was found to have a tree-in-bud pattern seen on CT scan, indicative of endobronchial spread of infection in the right upper lobe. The tree-in-bud pattern represents small pulmonary nodules or masses in the secondary pulmonary lobules and is often seen in conditions like tuberculosis. Bronchoscopy revealed white granulation tissue, confirming endobronchial tuberculosis as the cause in this patient.
This document discusses the approach to interstitial lung diseases (ILD) and diffuse parenchymal lung diseases (DPLD). It begins by reviewing the spectrum of ILD and DPLD, identifying clues from clinical presentation to make a diagnosis, and reviewing common radiographic findings. Key points include that ILD involves the pulmonary interstitium located between the epithelial and endothelial basement membranes. Clinical presentation of DPLD/ILD often involves dyspnea, cough, and abnormal chest imaging. Diagnosis involves considering history, physical exam, pulmonary function tests, imaging like chest radiographs and CT, and tissue sampling. Management depends on the specific diagnosis but may include treatments like corticosteroids, immunosuppressants, anti
This document discusses various asthma phenotypes or endotypes that have been identified based on differences in clinical characteristics, biomarkers and treatment responses. The two main endotypes discussed are TH2-high asthma and non-TH2 asthma. TH2-high asthma includes early-onset allergic asthma, late-onset eosinophilic asthma and exercise-induced asthma. It is characterized by eosinophilia, TH2 biomarkers and good response to corticosteroids and anti-TH2 targeted therapies. Non-TH2 asthma includes obesity-related asthma, neutrophilic asthma and smoking asthma. It has fewer clinical allergies and TH2 biomarkers, and poorer responses to corticosteroids. Distinct clinical features, genetics,
This document discusses various ventilatory modes and settings used in non-invasive ventilation (NIV). It begins by explaining the unique characteristics of NIV, namely the non-hermetic nature of the system due to leaks, and the variable resistance of the upper airway. It then describes common ventilator modes for NIV including spontaneous, spontaneous-timed, pressure assist control, and timed modes. The document outlines optimal settings for various parameters such as inspiratory and expiratory pressure, pressure support, rise time, inspiratory time and cycling criteria. It emphasizes titrating settings to achieve adequate ventilation while minimizing patient effort and discomfort.
This document discusses bronchial hyperresponsiveness and bronchial provocation tests. It begins by defining asthma as a chronic inflammatory airway disease characterized by variable airflow obstruction and hyperresponsiveness to triggers. Bronchial hyperresponsiveness is an abnormal increase in airflow limitation following exposure to a stimulus and can be quantified using bronchial provocation tests. Several types of direct and indirect stimuli are described for use in bronchial provocation tests, with methacholine challenge being the most commonly used direct stimulus test due to its safety and sensitivity. The document outlines the procedures, interpretations, and indications for various bronchial provocation tests.
Development of lung and related abnormalitiesayush jain
1) The lung develops from the ventral wall of the foregut starting in the 4th week of gestation. The respiratory diverticulum forms the trachea and bronchi through branching morphogenesis.
2) There are four main stages of lung development - embryonic, pseudoglandular, canalicular, and saccular. Important events include formation of the air-blood barrier and surfactant production.
3) At birth, the alveolar stage begins as babies transition to breathing air. Alveoli continue developing postnatally, with most forming in the first 2 years of life. Abnormalities can occur if development is disrupted, such as tracheoesophageal fistula.
This document discusses idiopathic pulmonary fibrosis (IPF), a chronic, progressive fibrosing interstitial pneumonia of unknown cause associated with a histopathologic pattern of usual interstitial pneumonia (UIP). It defines IPF and outlines the diagnostic criteria, which involves ruling out known causes, abnormal pulmonary function tests, characteristic radiologic findings on high-resolution computed tomography (HRCT), and surgical lung biopsy showing UIP pattern. HRCT features that are consistent and inconsistent with UIP are described. Guidelines for management of IPF are provided, including recommendations for pirfenidone and nintedanib based on recent clinical trials. Lung transplantation is the only treatment that increases long-term survival for patients with IPF.
This document discusses the anatomy and function of the oculomotor nerve (cranial nerve 3). It describes the nucleus and course of the nerve, causes of lesions, clinical features of total oculomotor nerve palsy, treatment options which may include surgery or monitoring, and differential diagnoses. History, examination, and investigations are outlined to evaluate patients presenting with oculomotor nerve palsies.
This document provides information on diffuse parenchymal lung disease (DPLD) and idiopathic interstitial pneumonias (IIPs). It begins with an overview of common IIPs including idiopathic pulmonary fibrosis (IPF), other IIPs, familial IIP, IIP with autoimmune features, and smoking-related ILDs. It then discusses diagnosing other ILDs through clinical, radiological findings and management approaches. Specific ILDs covered include CTD-associated ILDs, diffuse cystic lung diseases like lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, pulmonary alveolar proteinosis, and diffuse alveolar damage
Based on the information provided:
- The patient has a UIP pattern on HRCT consistent with IPF.
- His occupational exposure to asbestos 35 years ago could be contributing to the fibrosis.
- His rheumatoid arthritis is seronegative so unlikely the cause.
- A multidisciplinary discussion including review of HRCT, pulmonary function tests and clinical history is needed to determine if he meets criteria for a confident diagnosis of IPF. Given his occupational exposure, other ILDs need to be considered or excluded as well.
ABPA is a complex immunological lung disorder caused by hypersensitivity to Aspergillus fumigatus antigens in patients with asthma or cystic fibrosis. It presents as poorly controlled asthma, bronchiectasis, or recurrent lung infiltrates. Diagnosis requires elevated total IgE levels, positive skin test or specific IgE to A. fumigatus, and meeting two of three criteria including precipitating antibodies, chest imaging findings, or eosinophil count. Treatment involves oral steroids and long-term antifungal therapy to reduce IgE levels and control symptoms. The disease has a relapsing course and complications include worsening asthma and permanent lung damage if not treated early.
The document discusses interstitial lung disease (ILD), including its common features, types, causes, diagnostic approach and treatment. It describes various ILD types such as idiopathic pulmonary fibrosis and sarcoidosis. Imaging and biopsy are used to diagnose ILD and determine prognosis. Treatment involves identifying and removing environmental causes, suppressing inflammation, and managing complications like right heart failure.
The document discusses various COPD phenotypes including:
1) Asthma-COPD overlap phenotype characterized by incompletely reversible airway obstruction and asthma-like features.
2) Frequent exacerbator phenotype defined as 2 or more exacerbations per year which increases health risks.
3) Upper lobe-predominant emphysema phenotype where surgical lung volume reduction may help.
4) Infrequent exacerbator phenotype experiencing less than two exacerbations per year requiring only bronchodilators.
5) Alpha-1 antitrypsin deficiency phenotype which is a genetic cause of panlobular emphysema.
FlashPath - Lung - Cryptogenic Organizing Pneumonia (Bronchiolitis Obliterans...Hazem Ali
This document discusses cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia (BOOP). It presents the following key points:
1. COP is characterized by subacute onset of respiratory symptoms and radiologic evidence of patchy consolidation and nodularity. Histologically, it shows intraluminal plugs of granulation tissue (Masson bodies) in the respiratory bronchioles and alveolar ducts.
2. COP has an excellent prognosis and is usually steroid responsive. However, steroid resistance or relapse may occur after treatment is stopped.
3. The pathology of COP must be differentiated from other interstitial lung diseases that present similarly such as usual inter
Practical approach to Idiopathic Pulmonary Fibrosis.Hiba Ashibany
This document provides information on idiopathic pulmonary fibrosis (IPF), including its causes, diagnosis, clinical features, prognosis, and treatment approaches. It summarizes that IPF is a progressive lung disease of unknown cause where scarring develops in the lungs. Diagnosis involves ruling out other conditions, imaging, and sometimes biopsies. Prognosis is generally poor with median survival of 3 years. Treatment includes drugs like pirfenidone and nintedanib that can slow disease progression in mild to moderate IPF.
This document summarizes various connective tissue disease-associated interstitial lung diseases. It describes common intrathoracic manifestations and imaging findings for conditions like rheumatoid arthritis, progressive systemic sclerosis, systemic lupus erythematosus, and polymyositis/dermatomyositis. For each condition, it lists typical radiological patterns seen on CT such as ground glass opacities, reticulation, consolidation, and honeycombing. Photomicrographs are also included to illustrate histopathological findings for some of the interstitial lung diseases.
IPF AND PROGRESSIVE PULMONARY FIBROSIS 2022UPDATE(ATS.pptxAnjanaAnilkumar14
This document provides updates on the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) from the ATS/ERS:
- For IPF, a conditional recommendation was made for transbronchial lung cryobiopsy as an alternative to surgical lung biopsy. No recommendation was given for or against genomic testing. Antacid medications and antireflux surgery are not recommended for treating IPF.
- PPF is defined as worsening symptoms, radiological progression, and physiological progression within a year in an ILD other than IPF with no alternative explanation. A conditional recommendation was made for nintedanib for PPF, and more research on pirfenidone
This document provides a summary of an presentation on approaches to interstitial lung disease (ILD) and updates in idiopathic pulmonary fibrosis (IPF) management. It begins with an introduction to ILDs and the pulmonary interstitium. It then covers the pathogenesis, classification, epidemiology, clinical assessment including history, exams, tests and tissue sampling, and radiological and pathological findings of ILDs. A significant portion discusses IPF specifically, including prognosis, guidelines for diagnosis, and medical therapies including pirfenidone and nintedanib which have been shown to reduce lung function decline in clinical trials. It concludes with experience using pirfenidone in Saudi Arabia.
Pulmonary sarcoidosis is a multisystem inflammatory disease of unknown etiology characterized by non-caseating granulomas. It most commonly affects the lungs, skin, eyes and lymph nodes. The pathogenesis involves accumulation of inflammatory cells and T lymphocytes forming granulomas that can damage tissues. Diagnosis is based on clinical features, radiological evidence of non-caseating granulomas on biopsy with other causes excluded. Treatment depends on severity and organ involvement but may include corticosteroids.
This document discusses pulmonary function tests (PFTs), including their goals, uses, limitations, procedures, and interpretations. PFTs are used to assess lung function before surgeries and characterize any pulmonary dysfunction. Key information obtained from PFTs includes measurements of forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), their ratio (FEV1/FVC), and peak expiratory flow rate. Interpretations of these values can indicate restrictive or obstructive lung disease. The document outlines how PFTs are performed using portable devices or clinic spirometers and flow-volume loops.
This document describes a case of a 48-year-old male admitted with hemoptysis and cough who was found to have a tree-in-bud pattern seen on CT scan, indicative of endobronchial spread of infection in the right upper lobe. The tree-in-bud pattern represents small pulmonary nodules or masses in the secondary pulmonary lobules and is often seen in conditions like tuberculosis. Bronchoscopy revealed white granulation tissue, confirming endobronchial tuberculosis as the cause in this patient.
This document discusses the approach to interstitial lung diseases (ILD) and diffuse parenchymal lung diseases (DPLD). It begins by reviewing the spectrum of ILD and DPLD, identifying clues from clinical presentation to make a diagnosis, and reviewing common radiographic findings. Key points include that ILD involves the pulmonary interstitium located between the epithelial and endothelial basement membranes. Clinical presentation of DPLD/ILD often involves dyspnea, cough, and abnormal chest imaging. Diagnosis involves considering history, physical exam, pulmonary function tests, imaging like chest radiographs and CT, and tissue sampling. Management depends on the specific diagnosis but may include treatments like corticosteroids, immunosuppressants, anti
This document discusses various asthma phenotypes or endotypes that have been identified based on differences in clinical characteristics, biomarkers and treatment responses. The two main endotypes discussed are TH2-high asthma and non-TH2 asthma. TH2-high asthma includes early-onset allergic asthma, late-onset eosinophilic asthma and exercise-induced asthma. It is characterized by eosinophilia, TH2 biomarkers and good response to corticosteroids and anti-TH2 targeted therapies. Non-TH2 asthma includes obesity-related asthma, neutrophilic asthma and smoking asthma. It has fewer clinical allergies and TH2 biomarkers, and poorer responses to corticosteroids. Distinct clinical features, genetics,
This document discusses various ventilatory modes and settings used in non-invasive ventilation (NIV). It begins by explaining the unique characteristics of NIV, namely the non-hermetic nature of the system due to leaks, and the variable resistance of the upper airway. It then describes common ventilator modes for NIV including spontaneous, spontaneous-timed, pressure assist control, and timed modes. The document outlines optimal settings for various parameters such as inspiratory and expiratory pressure, pressure support, rise time, inspiratory time and cycling criteria. It emphasizes titrating settings to achieve adequate ventilation while minimizing patient effort and discomfort.
This document discusses bronchial hyperresponsiveness and bronchial provocation tests. It begins by defining asthma as a chronic inflammatory airway disease characterized by variable airflow obstruction and hyperresponsiveness to triggers. Bronchial hyperresponsiveness is an abnormal increase in airflow limitation following exposure to a stimulus and can be quantified using bronchial provocation tests. Several types of direct and indirect stimuli are described for use in bronchial provocation tests, with methacholine challenge being the most commonly used direct stimulus test due to its safety and sensitivity. The document outlines the procedures, interpretations, and indications for various bronchial provocation tests.
Development of lung and related abnormalitiesayush jain
1) The lung develops from the ventral wall of the foregut starting in the 4th week of gestation. The respiratory diverticulum forms the trachea and bronchi through branching morphogenesis.
2) There are four main stages of lung development - embryonic, pseudoglandular, canalicular, and saccular. Important events include formation of the air-blood barrier and surfactant production.
3) At birth, the alveolar stage begins as babies transition to breathing air. Alveoli continue developing postnatally, with most forming in the first 2 years of life. Abnormalities can occur if development is disrupted, such as tracheoesophageal fistula.
This document discusses idiopathic pulmonary fibrosis (IPF), a chronic, progressive fibrosing interstitial pneumonia of unknown cause associated with a histopathologic pattern of usual interstitial pneumonia (UIP). It defines IPF and outlines the diagnostic criteria, which involves ruling out known causes, abnormal pulmonary function tests, characteristic radiologic findings on high-resolution computed tomography (HRCT), and surgical lung biopsy showing UIP pattern. HRCT features that are consistent and inconsistent with UIP are described. Guidelines for management of IPF are provided, including recommendations for pirfenidone and nintedanib based on recent clinical trials. Lung transplantation is the only treatment that increases long-term survival for patients with IPF.
This document discusses the anatomy and function of the oculomotor nerve (cranial nerve 3). It describes the nucleus and course of the nerve, causes of lesions, clinical features of total oculomotor nerve palsy, treatment options which may include surgery or monitoring, and differential diagnoses. History, examination, and investigations are outlined to evaluate patients presenting with oculomotor nerve palsies.
This document provides information on diffuse parenchymal lung disease (DPLD) and idiopathic interstitial pneumonias (IIPs). It begins with an overview of common IIPs including idiopathic pulmonary fibrosis (IPF), other IIPs, familial IIP, IIP with autoimmune features, and smoking-related ILDs. It then discusses diagnosing other ILDs through clinical, radiological findings and management approaches. Specific ILDs covered include CTD-associated ILDs, diffuse cystic lung diseases like lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, pulmonary alveolar proteinosis, and diffuse alveolar damage
Based on the information provided:
- The patient has a UIP pattern on HRCT consistent with IPF.
- His occupational exposure to asbestos 35 years ago could be contributing to the fibrosis.
- His rheumatoid arthritis is seronegative so unlikely the cause.
- A multidisciplinary discussion including review of HRCT, pulmonary function tests and clinical history is needed to determine if he meets criteria for a confident diagnosis of IPF. Given his occupational exposure, other ILDs need to be considered or excluded as well.
The CT scan shows bilateral, basal-predominant reticular opacities and honeycombing. Given the patient's history of asbestos exposure, though brief, the radiological findings are most consistent with a diagnosis of asbestosis. Asbestosis is the correct answer.
Interstitial lung disease (ILD) is a group of disorders causing scarring of the lungs. Idiopathic pulmonary fibrosis is the most common ILD, accounting for around half of cases. It generally affects older adults and smokers and causes shortness of breath, cough, and lung function decline. Diagnosis involves imaging, pulmonary function tests, and biopsy. Approved treatments are pirfenidone and nintedanib, which can slow disease progression, but prognosis remains poor with median survival of 3-4 years. Nonspecific interstitial pneumonia is another ILD that may be idiopathic or associated with connective tissue diseases.
1) The patient presents with a history of recurrent chest infections and inspiratory crackles on examination. Imaging and pulmonary function tests are required to diagnose interstitial lung disease.
2) Idiopathic pulmonary fibrosis is a chronic, progressive form of interstitial lung disease of unknown cause characterized by fibrosis of the lungs. It carries a poor prognosis with median survival of 3 years.
3) Diagnosis requires ruling out other causes through history, imaging showing reticular opacities and honeycombing, and lung biopsy if imaging is not definitive. Treatment focuses on managing complications, vaccination, oxygen therapy and consideration of lung transplantation in advanced cases.
Interstitial lung disease (ILD) is a group of diseases causing fibrosis in the lungs, leading to stiffness and difficulty in breathing and oxygen delivery to the bloodstream. This presentation gives an overview on "Diagnosis of ILD". For more information, please contact us: 9779030507.
This document provides an overview of interstitial lung disease (ILD), also known as diffuse parenchymal lung disease. It discusses the epidemiology, diagnostic approach, classification, and treatment of ILD. The diagnostic approach involves obtaining a thorough history, physical exam, pulmonary function tests, imaging like chest X-rays and HRCT, and tissue sampling via bronchoscopy or surgical lung biopsy. ILDs can be classified as idiopathic interstitial pneumonias, granulomatous diseases like sarcoidosis, connective tissue disease-associated, and those related to occupational or environmental exposures. Treatment depends on the underlying cause but may include immunosuppression, antifibrotic drugs, managing comorbid
This document discusses various idiopathic interstitial pneumonias (IIPs), including their definitions, histological features, radiographic appearances, treatments, and prognoses. It covers common IIPs such as idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), and cryptogenic organizing pneumonia (COP). Lung biopsy is an important tool to distinguish between IIPs and make treatment decisions, but larger tissue samples are often needed due to sampling errors with transbronchial biopsies
Idiopathic Interstitial Pneumonia With Autoimmune Features(IPAF).pptxKefelegnNathan1
1) A 35-year-old female patient presented with shortness of breath and generalized body swelling for 1 month. She also had orthopnea and productive cough for the same duration.
2) Examination found bibasilar crackles, accentuated pulmonary component of S2, and echocardiogram showed severe pulmonary hypertension and RV dysfunction.
3) Investigations revealed elevated RF, ANA 1:100, diffuse bilateral pulmonary fibrosis and fibrosing mediastinitis on CT scan. A diagnosis of IPAF was made.
This document provides an overview of interstitial lung disease (ILD). ILD encompasses over 200 lung disorders that involve scarring or damage to the lungs' interstitium. Progressive fibrosis can occur in some ILDs and is associated with worse outcomes. Idiopathic pulmonary fibrosis is the most common progressive ILD and is characterized by lung scarring. Progressive-fibrosing ILD describes patients with fibrotic ILDs that may deteriorate despite treatment. Diagnosis involves evaluating symptoms, imaging, pulmonary function tests, biopsies and labs to identify the specific ILD and develop a treatment plan which may include immunosuppressants or removing environmental exposures.
This document provides an overview of connective tissue disease (CTD)-associated interstitial lung disease (ILD). Some key points:
- ILD is a common pulmonary complication in patients with CTDs like systemic sclerosis (SSc), rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE). It can occur concurrently with or after diagnosis of the CTD.
- The pathogenesis involves autoimmune mechanisms, genetic factors, environmental exposures, and inflammatory cytokines that cause lung inflammation and fibrosis.
- SSc has the highest rate of ILD of all CTDs, affecting 40-80% of patients. Antibodies to topoisomerase I are associated with increased
This document discusses the diagnosis of interstitial lung disease (ILD). It defines ILD as a collection of over 100 lung disorders that share clinical, radiographic, and pathological features. ILD is classified based on patterns seen on histology and radiography. Risk factors include age, smoking, occupation, and family history. Signs and symptoms include dyspnea, cough, chest pain, and digital clubbing. Diagnostic tests involve pulmonary function tests, chest imaging like HRCT, bronchoscopy, and surgical lung biopsy. HRCT is more sensitive than chest x-rays and can identify patterns like ground glass opacities and cysts that indicate different diseases.
The document discusses diffuse parenchymal lung diseases (DPLDs), a heterogeneous group of conditions that affect the lungs. Key points:
- DPLDs include idiopathic pulmonary fibrosis, which has characteristic radiologic and histologic patterns. It typically presents with progressive breathlessness.
- Sarcoidosis is a multisystem granulomatous disorder characterized by non-caseating granulomas. It commonly causes bilateral hilar lymphadenopathy and lung infiltrates.
- Pulmonary eosinophilia refers to lung abnormalities and blood eosinophilia. It includes conditions like Churg-Strauss syndrome and acute eosinophilic pneumonia.
New ulmonary arterial hypertension in rheumatic diseases 財團法人風濕病基金會台灣抗風濕病聯盟
This document summarizes a presentation on pulmonary artery hypertension (PAH) in rheumatic diseases. It begins with a case presentation of a patient diagnosed with limited systemic sclerosis and PAH who was treated with various medications. It then provides background on PAH classification and the pathophysiology of PAH in connective tissue diseases. Specifically, it discusses the prevalence of PAH in different rheumatic diseases like systemic sclerosis, the mechanisms involved in pathogenesis, and differences in phenotypes between SSc-PAH and non-SSc PAH. Treatment approaches are also summarized.
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Children's interstitial lung disease (chILD) involves the alveoli and surrounding tissues, leading to gas exchange issues. It was previously called interstitial lung disease but diffuse infiltrative lung disease is a more accurate term. Common causes of chILD include surfactant dysfunction disorders, lung growth abnormalities, neuroendocrine cell hyperplasia of infancy, and pulmonary interstitial glycogenosis. Diagnosis involves clinical evaluation, imaging like HRCT, pulmonary function tests, and sometimes lung biopsy. Management is generally supportive care though corticosteroids may help in some cases. Prognosis depends on the underlying condition but some forms of chILD have high mortality.
Pulmonary Hypertension for general physicians Sarfraz Saleemi
This patient, a 30-year-old woman, presented with progressive shortness of breath on exertion for two years. Tests revealed her pulmonary artery pressure was elevated at 55 mm Hg. Right heart catheterization confirmed a diagnosis of pulmonary arterial hypertension, showing a mean pulmonary artery pressure of 66 mm Hg and pulmonary vascular resistance of 15 wood units. As treatment for this non-vasoreactive pulmonary arterial hypertension, she will begin oral combination therapy based on her intermediate risk status.
The document provides information on the Global Initiative for Chronic Obstructive Lung Disease (GOLD) including its objectives to increase awareness of COPD, improve diagnosis and management, and stimulate research. It defines COPD as a preventable disease characterized by airflow limitation caused by an abnormal inflammatory response to noxious particles. The document also outlines the classification of COPD severity based on lung function tests, risk factors, pathogenesis, management approaches, and goals of reducing symptoms and disease progression.
Clinical and radiological features of idiopathic interstitial pneumonias (IIP...CesarAugustoArroyo
The document provides a pictorial review of the clinical and radiological features of idiopathic interstitial pneumonias (IIPs) according to the updated 2013 ATS/ERS classification. IIPs are a group of diffuse parenchymal lung diseases characterized by inflammation and fibrosis of the interstitium. Major IIPs include idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP), and acute interstitial pneumonia (AIP). The diagnosis of IPF requires excluding other known causes and
This document provides an overview of key aspects of clinical research papers, including their typical structure and components. It outlines the main sections such as the title, abstract, introduction, methods, results, discussion, and references. It also describes important considerations for study design, including defining the study population and ensuring internal and external validity. Common study designs like randomized controlled trials and how to properly implement randomization and blinding are covered.
This document provides an overview of evidence-based medicine (EBM). It defines EBM as integrating the best available research evidence with clinical expertise and patient values. The key steps of EBM are outlined as formulating a clinical question using PICO (population, intervention, comparison, outcome), searching for evidence, appraising research studies, and applying the evidence to clinical problems. Study designs such as randomized controlled trials and systematic reviews are discussed. Methods for critically appraising studies including assessing validity and determining the clinical importance of results are also summarized.
1) The document discusses a lecture on evidence-based medicine (EBM) and critical appraisal.
2) EBM involves integrating the best available research evidence with clinical expertise and patient values. It includes formulating clinical questions, searching for evidence, appraising research, and applying the evidence to patient care.
3) The lecture reviews the principles of EBM and critical appraisal, including how to formulate answerable clinical questions using the PICO framework, search for evidence, and appraise different types of research studies.
This document provides an overview of scientific writing and research proposals. It discusses types of scientific publications such as journal articles, books, and conference posters. It emphasizes using clear, precise language and proper structure for scientific papers, including titles, introductions, methods, results, and references sections. The document also outlines the key elements of a good research proposal, such as stating the problem, reviewing previous literature, describing the methodology, presenting a timeline and budget, and listing references. Researchers are advised to write proposals that are coherent, informative, and clearly structured to convince readers of the significance and merit of the proposed research.
This document discusses novel treatment options for asthma, focusing on biologic-based targeted therapies. It summarizes the four approved type-2 targeted biologic therapies that target IL-5 and IgE, as well as IL-4 and IL-13. These target key pathways involved in type-2 inflammation like eosinophil recruitment and activation. Emerging therapies also target other inflammatory pathways like IL-17. Characterization of inflammatory biomarkers and phenotypes helps identify patients that may benefit most from specific targeted therapies.
1. The document provides an overview of evidence-based medicine (EBM) and the process of critically appraising research evidence. EBM involves integrating the best available research evidence with clinical expertise and patient values and preferences.
2. The key steps of EBM are outlined, including formulating a clear clinical question using PICO (population, intervention, comparison, outcome), searching for and appraising the evidence, and applying the results to the clinical problem.
3. Users' guides are provided for critically appraising different study designs, focusing on whether the results are valid and assessing the magnitude and precision of the treatment effect. Factors like randomization, blinding, follow-up, and equal treatment of groups
1. Transbronchial biopsy is the least invasive approach to obtain a histologic diagnosis for a 60-year-old man with shortness of breath, a history of smoking, and basilar crackles. Objective parameters like 6MWT, DLCO, FVC, and HRCT can assess progression of the disease. Lung transplantation is the best curative treatment.
2. A 50-year-old current smoker with shortness of breath and cough showing findings on HRCT and PFTs would be diagnosed with RB-ILD based on surgical lung biopsy findings.
3. A 40-year-old man with rapid deterioration and bilateral infiltrates on CXR would be diagnosed with acute eosin
Pneumonia can be categorized as community-acquired pneumonia (CAP), healthcare-associated pneumonia (HCAP), hospital-acquired pneumonia (HAP) including ventilator-associated pneumonia (VAP). HCAP refers to patients who received recent healthcare but did not stay overnight in the hospital. CAP occurs in people acquired in the community with an annual rate of 5.16 to 6.11 cases per 1000 persons increasing with age. Streptococcus pneumoniae is the most common worldwide cause of CAP. Pneumonia pathogens can be typical bacteria like S. pneumoniae or atypical organisms such as Legionella spp, Mycoplasma pneumoniae, and Chlamydophila pneumoniae.
This study analyzed 29 cases of Middle East Respiratory Syndrome Coronavirus (MERS-CoV) in Saudi Arabia from March to May 2014. Most cases were male Saudi nationals over age 40. Common symptoms were fever, cough and shortness of breath. Patients had abnormal chest imaging and laboratory abnormalities including low white blood cell count. Ten patients (34%) died, generally being older, male smokers with more severe symptoms and worse laboratory and blood gas values. MERS-CoV disproportionately affected health care workers through close contact with infected patients.
Neuromuscular Disorders Respiratory Complications and AssessmentNahid Sherbini
This document discusses respiratory complications and management in patients with neuromuscular disorders. Key points:
- Duchenne muscular dystrophy is the most common childhood muscular dystrophy, causing progressive muscle weakness.
- Respiratory muscle weakness can occur independently of peripheral muscle weakness and should be evaluated through tests like PFTs, MIP, MEP, and cough assessment.
- Non-invasive ventilation may benefit those requiring short term or intermittent support, while invasive ventilation is preferred for acute respiratory failure due to risks of NPPV. Proper respiratory management can extend lifespans.
Hemoptysis is defined as the spitting of blood from the lungs or bronchial tubes. It can be classified based on severity from mild to massive. Common causes include infections like tuberculosis, cancers, vascular abnormalities and vasculitis. Initial management focuses on airway protection, oxygenation and circulation. Bronchoscopy helps identify the bleeding site and allows local measures like lavage, vasoconstrictors and tamponade. For persistent or massive bleeding, bronchial artery embolization or surgery may be needed. Precise localization through CT and arteriography guides definitive treatment.
The national lung screening trial /Nahid SherbiniNahid Sherbini
The National Lung Screening Trial (NLST) compared low-dose CT screening to chest x-ray (CXR) screening for lung cancer in high-risk individuals. Over 53,000 participants were randomized to receive either low-dose CT or CXR screening annually for three years. The primary endpoint was lung cancer mortality. An interim analysis found that low-dose CT screening reduced lung cancer mortality by 20% compared to CXR, with fewer advanced stage cancers detected in the CT group. However, the false positive rate was high at around 95% for both screening methods.
Evaluation of preoperative pulmonary risk By Nahid SherbiniNahid Sherbini
- Pulmonary complications are a major cause of postoperative morbidity and mortality. The risk depends on patient-related factors like age, smoking history, COPD, asthma, obesity, sleep apnea, and heart failure as well as procedure-related factors like the surgical site and duration of anesthesia.
- A thorough preoperative evaluation involves reviewing the patient's history, performing a physical exam, and testing like arterial blood gases, chest x-ray, and pulmonary function tests to determine their risk level. Assigning a risk level helps guide risk reduction strategies in high risk patients.
- Pulmonary complications are a major cause of postoperative morbidity and mortality. The risk depends on patient-related factors like age, smoking history, COPD, asthma, obesity, sleep apnea, and heart failure as well as procedure-related factors like the surgical site and duration of anesthesia.
- A thorough preoperative evaluation involves reviewing the patient's history, performing a physical exam, and testing like arterial blood gases, chest x-ray, and pulmonary function tests to determine their risk level. Assigning a risk level helps guide risk reduction strategies in high risk patients.
Control of Ventilation /Lung Physiology by Nahid SherbiniNahid Sherbini
The document summarizes the neural control of breathing. It discusses how breathing is regulated by central neuronal networks in the brainstem and spinal cord to meet metabolic demands. The central neurons in the medulla and pons form the basic respiratory center that produces and controls respiration. These centers integrate input from higher brain areas, mechanoreceptors, and peripheral chemoreceptors. They regulate breathing frequency and tidal volume through motor neurons that control respiratory muscles. Peripheral chemoreceptors in the carotid bodies also influence breathing in response to changes in blood oxygen and carbon dioxide levels.
This document discusses asthma management during pregnancy. It begins by describing how pregnancy can affect asthma severity, noting that prior asthma severity predicts severity during pregnancy. It then explains how asthma can impact pregnancy outcomes like preterm delivery. The rest of the document covers general asthma care during pregnancy, including monitoring, education, trigger avoidance, and medications. It notes most asthma medications are category B or C but are generally considered safe in pregnancy with monitoring. Inhaled short-acting beta agonists are recommended for acute exacerbations. The goal is preventing acute episodes and optimizing lung function to reduce risks.
The document summarizes the neural control of breathing. It discusses how breathing is regulated by central neuronal networks in the brainstem and spinal cord to meet metabolic demands. The central neurons in the medulla and pons form the basic respiratory center that produces and controls respiration. These centers integrate input from higher brain areas, mechanoreceptors, and peripheral chemoreceptors. They regulate breathing frequency and tidal volume through motor neurons that control respiratory muscles. Chemical control of breathing also occurs through central and peripheral chemoreceptors that sense changes in blood gases like oxygen and carbon dioxide to modulate ventilation.
This document discusses asthma management during pregnancy. It begins by describing how pregnancy can affect asthma severity, noting that prior asthma severity predicts severity during pregnancy. It then explains how asthma can impact pregnancy outcomes like preterm delivery. The rest of the document covers general asthma care during pregnancy, including monitoring, education, trigger avoidance, and medications. It notes most asthma medications are category B or C but are generally considered safe in pregnancy with monitoring. Inhaled short-acting beta agonists are recommended for acute exacerbations. The goal is preventing acute episodes and optimizing lung function to reduce risks.
Basavarajeeyam is an important text for ayurvedic physician belonging to andhra pradehs. It is a popular compendium in various parts of our country as well as in andhra pradesh. The content of the text was presented in sanskrit and telugu language (Bilingual). One of the most famous book in ayurvedic pharmaceutics and therapeutics. This book contains 25 chapters called as prakaranas. Many rasaoushadis were explained, pioneer of dhatu druti, nadi pareeksha, mutra pareeksha etc. Belongs to the period of 15-16 century. New diseases like upadamsha, phiranga rogas are explained.
Here is the updated list of Top Best Ayurvedic medicine for Gas and Indigestion and those are Gas-O-Go Syp for Dyspepsia | Lavizyme Syrup for Acidity | Yumzyme Hepatoprotective Capsules etc
ABDOMINAL TRAUMA in pediatrics part one.drhasanrajab
Abdominal trauma in pediatrics refers to injuries or damage to the abdominal organs in children. It can occur due to various causes such as falls, motor vehicle accidents, sports-related injuries, and physical abuse. Children are more vulnerable to abdominal trauma due to their unique anatomical and physiological characteristics. Signs and symptoms include abdominal pain, tenderness, distension, vomiting, and signs of shock. Diagnosis involves physical examination, imaging studies, and laboratory tests. Management depends on the severity and may involve conservative treatment or surgical intervention. Prevention is crucial in reducing the incidence of abdominal trauma in children.
Histololgy of Female Reproductive System.pptxAyeshaZaid1
Dive into an in-depth exploration of the histological structure of female reproductive system with this comprehensive lecture. Presented by Dr. Ayesha Irfan, Assistant Professor of Anatomy, this presentation covers the Gross anatomy and functional histology of the female reproductive organs. Ideal for students, educators, and anyone interested in medical science, this lecture provides clear explanations, detailed diagrams, and valuable insights into female reproductive system. Enhance your knowledge and understanding of this essential aspect of human biology.
Cell Therapy Expansion and Challenges in Autoimmune DiseaseHealth Advances
There is increasing confidence that cell therapies will soon play a role in the treatment of autoimmune disorders, but the extent of this impact remains to be seen. Early readouts on autologous CAR-Ts in lupus are encouraging, but manufacturing and cost limitations are likely to restrict access to highly refractory patients. Allogeneic CAR-Ts have the potential to broaden access to earlier lines of treatment due to their inherent cost benefits, however they will need to demonstrate comparable or improved efficacy to established modalities.
In addition to infrastructure and capacity constraints, CAR-Ts face a very different risk-benefit dynamic in autoimmune compared to oncology, highlighting the need for tolerable therapies with low adverse event risk. CAR-NK and Treg-based therapies are also being developed in certain autoimmune disorders and may demonstrate favorable safety profiles. Several novel non-cell therapies such as bispecific antibodies, nanobodies, and RNAi drugs, may also offer future alternative competitive solutions with variable value propositions.
Widespread adoption of cell therapies will not only require strong efficacy and safety data, but also adapted pricing and access strategies. At oncology-based price points, CAR-Ts are unlikely to achieve broad market access in autoimmune disorders, with eligible patient populations that are potentially orders of magnitude greater than the number of currently addressable cancer patients. Developers have made strides towards reducing cell therapy COGS while improving manufacturing efficiency, but payors will inevitably restrict access until more sustainable pricing is achieved.
Despite these headwinds, industry leaders and investors remain confident that cell therapies are poised to address significant unmet need in patients suffering from autoimmune disorders. However, the extent of this impact on the treatment landscape remains to be seen, as the industry rapidly approaches an inflection point.
Muktapishti is a traditional Ayurvedic preparation made from Shoditha Mukta (Purified Pearl), is believed to help regulate thyroid function and reduce symptoms of hyperthyroidism due to its cooling and balancing properties. Clinical evidence on its efficacy remains limited, necessitating further research to validate its therapeutic benefits.
Integrating Ayurveda into Parkinson’s Management: A Holistic ApproachAyurveda ForAll
Explore the benefits of combining Ayurveda with conventional Parkinson's treatments. Learn how a holistic approach can manage symptoms, enhance well-being, and balance body energies. Discover the steps to safely integrate Ayurvedic practices into your Parkinson’s care plan, including expert guidance on diet, herbal remedies, and lifestyle modifications.
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Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...Oleg Kshivets
Overall life span (LS) was 1671.7±1721.6 days and cumulative 5YS reached 62.4%, 10 years – 50.4%, 20 years – 44.6%. 94 LCP lived more than 5 years without cancer (LS=2958.6±1723.6 days), 22 – more than 10 years (LS=5571±1841.8 days). 67 LCP died because of LC (LS=471.9±344 days). AT significantly improved 5YS (68% vs. 53.7%) (P=0.028 by log-rank test). Cox modeling displayed that 5YS of LCP significantly depended on: N0-N12, T3-4, blood cell circuit, cell ratio factors (ratio between cancer cells-CC and blood cells subpopulations), LC cell dynamics, recalcification time, heparin tolerance, prothrombin index, protein, AT, procedure type (P=0.000-0.031). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and N0-12 (rank=1), thrombocytes/CC (rank=2), segmented neutrophils/CC (3), eosinophils/CC (4), erythrocytes/CC (5), healthy cells/CC (6), lymphocytes/CC (7), stick neutrophils/CC (8), leucocytes/CC (9), monocytes/CC (10). Correct prediction of 5YS was 100% by neural networks computing (error=0.000; area under ROC curve=1.0).
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Dpld board reveiw 2019
1. DIFFUSE PARENCHYMAL LUNG
DISEASE
Dr Nahid Sherbini
Internal Medicine & Pulmonary Consultant
Certified from Harvard Medical School in Practice of clinical Research
2. LearningTargets -I-
•To elaborate the general diagnostic approach to
ILDs and Classifications
•To diagnose IPF and understand treatment
options
•To recognize forms of IIP and clinical relevance
3. DPLD I
Idiopathic Interstitial Pneumonias (IIPs)
• IPF
• Other IIPs
• Familial IP
• IP with autoimmune features (IPAF)
• Smoking-related ILDs
6. Introduction
(ILDs) are a heterogeneous group of
disorders that are classified together
because of similar clinical, radiographic,
physiologic, or pathologic manifestations .
8. What is the Pulmonary Interstitium?
•between the epithelial and
endothelial basement
membrane
•Expansion of the interstitial
compartment by
inflammation with or
without fibrosis
• Necrosis
• Hyperplasia
• Collapse of basement
membrane
• Inflammatory cells
9. Pathogenesis
The pathogenesis of ILDs is unknown.
But more and more facts have shown that
immune cells and their cytokines play an important
role in the course of ILDs.
20. Physical examinations
•Bilateral basilar, crepitant velcro-like rale
•wheezing, rhonchi and coarse rales are occasionally
heard
•with advanced disease, patients may have tachypnea
and tachycardia
•At last, pulmonary hypertention and cor pulmonale
may be exist
26. Q. 1
Classical HRCT findings for IPF , All true except :
a. Traction Bronchiectasis
b. Basal , Sub pleural , Central
c. Honeycombing
d. Reticular Pattern
27. Images courtesy of W. Richard Webb, MD.
Basal and peripheral reticulation
Reduced lung volume
38. DD
W (Wegner’s)
E (EP)
B (BOOP) COP
A (PAP, Aspiration)
L (Lymphoma)
L (Lipoid Pneumonia)
S (Sacroidosis)
39. Q. 2
55 year male with history of SOB and dry cough over 6 months. He
smokes occasionally . PFT Restrictive pattern with reduced capacity .
HIS HRCT show GGO with traction bronchiectasis .
Which of the following is the most likely diagnosis?
A. NSIP
B. UIP
C. RB-ILD
D. LIP
40. Q.3
In interstitial lung diseases, lung function tests most often show:
A. Reduced carbon monoxide diffusing capacity (DLCO)
B. Increased total lung capacity (TLC)
C. Airflow obstruction
D. Elevated arterial PCO2
41. PFT
•A restrictive defect :
(TLC), (FRC), (RV) ,(FVC) and (FEV1)
but usually the changes are in proportion to the
decreased lung volumes
Low DLCO
42. PFT
• A reduction (DLCO) is a common, but nonspecific finding in ILD- , the
severity of the DLCO reduction does not correlate well with disease
prognosis, unless the DLCO is less than 35 %.
• Due to effacement of the alveolar capillary units but more importantly, to
the extent of mismatching of ventilation and perfusion of the alveoli.
• In some ILDs, i.e sarcoidosis , there can be considerable reduction in lung
volumes and/or severe hypoxemia but normal or only slightly reduced
DLCO
43. Moderate to severe reduction of DLCO in the presence of
normal lung volumes in a patient with ILD suggests one
of the following:
a. Combined emphysema and ILD
b. Combined ILD and PVD
c. Pulmonary Langerhans cell histiocytosis
d. Pulmonary lymphangioleiomyomatosis
e. All of the above
Q.4
44. Q. 5
An interstitial pattern onCXR accompanied by obstructive airflow
suggestive of :
a. Sarcoidosis
b. Diffuse alveolar hemorrhage
c. Pulmonary lymphoangioliomatosis
d. Combined COPD and ILD
e. All of the above
45. An interstitial pattern on CXR accompanied by obstructive
airflow suggestive of :
• Sarcoidosis
• Lymphangioleiomyomatosis
• HP
• PLCH
• Combined COPD and ILD
46. 6MWT
•6MWT have correlated with prognosis in several studies of IPF
• Pulse oximetry desaturation to 88 during the 6MWT is
associated with a median survival of 3.21 y compared with a
median survival of 6.63 y in those who did not desaturate
below 89%.
47. Q. 6
Which of the following is false regarding Pulmonary hypertension in
ILDs ?
A.The cause of PH in ILD is likely multifactorial.
b.There is a linear correlation between PFT and PH in ILD.
C. Genetic predisposition not play a role
D. Proposed pathogenesis include presence of vaso-dilation
,angiopathy and PE
E.All are False
48. The cause of PH in ILD is likely multifactorial
The absence of a linear correlation between PFT
PH in ILD suggests that other factors may play a role ,These include the
following:
1.Vasoconstriction and vascular remodeling;
2.Perivascular fibrosis and vascular destruction;
3.Hypoxemia, both nocturnal and exertional;
4.Thrombotic angiopathy and pulmonary emboli;
5.Elevated pulmonary capillary wedge pressure resulting from peripheral
vascular occlusive disease, which has been described in both IPF and
sarcoidosis and/or diastolic dysfunction;
6.Microvascular inflammation and injury;
7.Pathobiological process (ie, vascular granulomas in sarcoidosis, PH of
Langerhan's cell histiocytosis); and
8.Genetic predisposition and varying gene expression
49. Q. 7
Which is true about BAL in ILDs ?
a. BAL is less likely to be helpful in patients with a radiographic
pattern suggestive of IPF.
b. BAL does not have an established role in the assessment of ILD
progression or response to therapy
c. Consist normally of macrophages >85%, lymphocytes 10-15%,
neutrophils ≤3%, eosinophils ≤1%, epithelial ≤5%
d. High CD4 /CD8 ratio in sarcoidosis and rheumatoid lung while
reveals low ratio in HP .
e. All are true
50. Q.
Which of the following associated with neutrophilicCellular pattern
in BAL ?
A. IPF
B. HP
C. COP
D. Drug induced Pneumonitis
E. Radiation Pneumonitis
51. ROLE OF BRONCHOALVEOLAR
LAVAGE (BAL)
•The lavage fluid is sent for cell counts, cultures for
mycobacterial, viral and fungal pathogens, and cytological
analysis.
•Virtually all patients presenting with hemoptysis and
radiographic ILD should undergo BAL to confirm an alveolar
source of bleeding and identify any infectious etiologies.
54. Q. 8
Which is true regardingVATS use for diagnosing ILDs ?
A. Low diagnostic accuracy
B. More morbidity and mortality than open lung biopsy
C. Role of BAL andTBBx is highly diagnostic in all IIP
D. Ideal biopsy include two or more surgical wedge biopsies with
areas of normal lung and samples should measure 3-5 cm in length
and 2-3 cm in depth
E. None of the above
55. Video AssistedThoracic Surgery (VATS)
ChangAC, et al. AnnThorac Surg. 2002.74;1942-1946.
Rena O, et al. Eur JCardiothorac Surg. 1999;16:624-627.
• VATS is the preferred procedure for obtaining a lung biopsy
High diagnostic accuracy
Less morbidity and mortality than open lung biopsy
BAL andTBBx limited to excluding other IPF mimickers
• Ideal biopsy
Two or more surgical wedge biopsies with areas of normal lung
Samples should measure 35 cm in length and 23 cm in depth
• Outpatient thoracoscopic lung biopsy can be a safe and effective
procedure for patients with interstitial or focal lung disease
Diagnosis obtained in 61/62 patients
72.5 % discharged home within 8 hours
22.5% discharged home within 23 hours
ATS/ERSConsensus Statement. AmJ Respir Crit Care Med. 2000;161:646-664.
56. Probability of Histologic Diagnosis of Diffuse Diseases
Surgical
Biopsy
1. Granulomatous diseases
2. Malignant tumors/lymphangitic
3. DAD (any cause)
4. Certain infections
5. Alveolar proteinosis
6. Eosinophilic pneumonia
7.Vasculitis
8. Amyloidosis
9. EG/HX/PLCH
10. LAM
11. RB/RBILD/DIP
12. UIP/NSIP/LIP COP
13. Small airways disease
14. PHT and PVOD
Often
Sometimes
Rare
Transbronchial
Biopsy
Courtesy of Kevin O. Leslie, MD.
60. Q. 9
• 55 y old , 30 y pack history
• Progressive dyspnea and cough
• Was working in plastic factory for
the last 30 y
• Bilateral infiltrate in chest
radiograph and cyst
• Surgical biopsy shown
What is true about the nature of the
disease ?
a.This stage carry good prognosis
b. Respond to Steroids
c. Changing his work will be
beneficial
d. Poor Prognosis
61. Q.
• 55 y old , 30 y pack history
• Progressive dyspnea and cough
• Was working in plastic factory for
the last 30 y
• Bilateral infiltrate in chest
radiograph and cyst
• Surgical biopsy shown
What is true about the nature of the
disease ?
a.This stage carry good prognosis
b. Respond to Steroids
c. Changing his work will be
beneficial
d. Poor Prognosis
• Fibrosis with honeycombing
• Architectural destruction
• Peripheral and basal distribution
• Patchy (i.e. normal and abnormal lung)
• Fibroblastic foci UIP
63. Average survival diagnosis of IPF is
approximately 2.5–3.5 years1 from diagnosis
Onset of symptoms
Initial visit
Kaplan-Meier plot of the survival
probability in IPF patients (n=238)2
1. Ley B et al.Am J Respir Crit Care Med 2010 October 8 2. KingTE et al. Am J Respir Crit Care Med 2001; 164: 1171-1181
65. Q .10
Which of the following conditions cause UIP pattern in
HRCT ?
a. IPF
b. Chronic HP
c. Drug Induced
d. Infections e.gTB
e. All of the above
66. Establish Diagnosis
Multi-Disciplinary Team (MDT)
Discussion
Clinical
• Symptoms
• Smoking history
• Exposures
• Features of CTD
• Examination
Investigations
• CXR
• CTThorax
• Blood tests
• Lung Function
Pathology
• Bronchoalveolar
lavage
• Surgical lung biopsy
67. Q.11
A confident diagnosis of idiopathic pulmonary fibrosis
(IPF) requires which one of the following?
A. Surgical lung biopsy
B. Usual interstitial pneumonia (UIP) pattern on lung
biopsy or HRCT.
C. Failure to respond to corticosteroid therapy
D. Evidence of disease progression
68. To Diagnose
•1. Exclude identifiable causes of ILD (e.g.,
occupational or environmental exposures, drugs
&radiation, CTDs)
•2. UIP pattern shown by: a) HRCT or b) Surgical
lung biopsy, in the absence of HRCT features
inconsistent with UIP Diagnostic Criteria for IPF
(ATS/ERS/JRS/ALAT statement. AJRCCM 2011)
69. Q. 12
73-year-old retired insulating engineer presents with a 6-m
history of increasing dyspnoea. He worked with asbestos for 2
years, 35 years ago. He has seronegative rheumatoid arthritis,
clubbing and basal crackles on chest examination.The CT scan
is shown below.
Which one of the following is the most likely diagnosis?
a. Idiopathic pulmonary fibrosis
B. Asbestosis
c. Rheumatoid lung
d. Lung adenocarcinoma
e. Bronchiectasis
70. A. Idiopathic pulmonary fibrosis
• Asbestosis is unlikely because the patient’s asbestos exposure was only
2 years in duration and his disease began more than 20 years later.The
absence of pleural plaques is evidence against asbestosis, in which
more than 95% of patients have pleural plaques demonstrable on chest
CT.
• Rheumatoid lung with interstitial fibrosis is unlikely in seronegative
disease, and clubbing is uncommon in rheumatoid pulmonary fibrosis.
• Lung adenocarcinoma remains a possible diagnosis but in this case is
less likely than IPF and the CT does not suggest the presence of a
cancer.
• Bronchiectasis is unlikely in the absence of cough and sputum
production and clubbing seldom occurs nowadays except in patients
with cystic. Bronchiectasis is not a prominent feature in the presented
CT.
71. Q 13
• 70 year-old never-smoking man, who is former office worker,
complains of a dry cough and progressive sob (NYHA class III) for
6 m. He takes 20 mg enalapril daily for hypertension. He has no
other diseases. He has not kept animals, or been exposed to dust
or fumes. Auscultation revealsVelcro rales over both lung bases.
There is no clubbing. Pulmonary function tests cannot be
performed because of impressive, possibly psychogenic,
hyperventilation.While breathing room air,ABG shows PaO2 72
mmHg, PaCO2 41 mmHg, pH 7.36 and SaO2 94%. His chest CT
image is shown below.
72. Q13
What is the initial
diagnostic test ?
a. Serology for CTD
b. VATS
c. BAL
d. TBB
e. Serum precipitating
Ab
73. Q 14
In patients with suspected idiopathic pulmonary fibrosis, the most
valuable measure is:
A. Bronchoscopy
B. ESR
C.Trial of steroids
D.Video-assisted thorascopic surgery (VATS)
E. None of the above
74. Q 15
75-year-old female is referred for dyspnoea on exertion and chronic cough
that have worsened progressively over the past 12 months. Pulmonary
function testing reveals an FVC of 72% predicted, FEV1 of 80% predicted
andTLCO of 38% predicted.The chest radiograph shows bilateral interstitial
basal infiltrates. On HRCT, bilateral reticular opacities and clustered basal
honeycombing are found. Open-lung biopsy reveals randomly distributed
foci of usual interstitial pneumonia surrounded by normal lung parenchyma.
What is the most appropriate therapy for this patient?
a. Pirfenidone
b. Bosentan
C. Acetylcysteine
d. Prednisolone/azathioprine
e. Supportive care
75.
76. The recommendation against the use of the
following agents for the treatment of IPF
• Anticoagulation
• Imatinib
• Combination prednisone, azathioprine, and N-
acetylcysteine
• Selective endothelin receptor antagonist (ambrisentan
• Phosphodiesterase-5 inhibitor (sildenafil)
• Dual endothelin receptor antagonists (macitentan,
bosentan)
(ATS/ERS/JRS/ALAT Guideline.AJRCCM 2015)
77. Q.
InAscend and CAPACITYTrial ,What is most common reported side
effect of Perfinidone compared to placebo?
A. Vomiting
B. Insomnia
C. Rash
D. Headache
E. Nausea
78. Q
Pooling the results ofAscend , Capacity 1 and Capacity 2 ,Which is
false ?
a. Improve dyspnoea
b. Reduce mortality
c. Reduce decline in 6 MWT
d. Decrease all cause mortality
e. Reduce decline in FVC
81. ASCEND: Key inclusion criteria
40–80 years of age
Definite UIP on HRCT, or possible UIP on HRCT plus definite or
probable UIP on surgical lung biopsy
Extent of fibrotic changes greater than extent of emphysema on
HRCT scan
FVC ≥50% and ≤90% predicted
DLCO ≥30% and ≤90% predicted
FEV1/FVC ratio ≥0.8
6MWT distance ≥150 m
King TE Jr, et al. N Engl J Med 2014;370:2083-2092.
84. TOMORROW: Annual rate of decline in
FVC
Difference between nintedanib 150 mg bid and placebo: p=0.064 vs placebo (pre-specified primary multiplicity-corrected
analysis [closed testing procedure]); p=0.014 vs placebo (pre-specified hierarchical testing procedure).
Richeldi L, et al. N Engl J Med 2011;365:1079–1087.
-0.19
-0.17
-0.21
-0.16
-0.06
-0.30
-0.25
-0.20
-0.15
-0.10
-0.05
0.00
Placebo
(n=83)
Nintedanib
50 mg qd
(n=85)
Nintedanib
50 mg bid
(n=86)
Nintedanib
100 mg bid
(n=85)
Nintedanib
150 mg bid
(n=84)
Annual
rate
of
FVC
decline,
L/year
[Mean
(SE)]
85. TOMORROW: Preservation of health-related
quality of life
5.46 4.67
2.18
1.48
-0.66
-4
-2
0
2
4
6
8
Placebo
(n=79)
Nintedanib
50 mg qd
(n=76)
Nintedanib
50 mg bid
(n=82)
Nintedanib
100 mg bid
(n=82)
Nintedanib
150 mg bid
(n=75)
Change
in
SGRQ
total
score
[Mean
(SE)]
*
*p=0.007 vs placebo.
SGRQ, St George’s Respiratory Questionnaire.
Richeldi L, et al. N Engl J Med 2011;365:1079–1087.
86.
87. All-cause mortality over 52 weeks: Pooled data
from INPULSIS®
Placebo
Nintedanib 150 mg bid
HR 0.70
(95% CI; 0.43, 1.12)
p=0.1399
Richeldi L, et al. N Engl J Med 2014;370:2071–2082.
89. Q 16
About GERD in patients with IPF ,Which of the
following statement correct ?
a. GERD is common (60-90%) in IPF
b. Majority (50-75%) asymptomatic.
c. May contribute to fibrosis progression, AE.
d. Some studies suggest use of GERD medications to be
an independent predictor of longer survival time in IPF,
associated with slower decline in FVC, decreasedAE.
e. All are true
90. Treatment of IPF
• “We suggest that clinicians use regular antacid
treatment for patients with IPF.”
• “ lung transplantation in patients with IPF.”
• “The committee did not make a recommendation
regarding treatment of PH in patients with IPF.”
(ATS/ERS/JRS/ALAT Guideline. AJRCCM 2015)
91. Q 17
Which of the following is true regarding NSIP?
A. NSIP is a specific disease entity.
B. Prognosis associated with NSIP and UIP are
similar.
C. NSIP is commonly associated with
connective tissue diseases.
D. NSIP commonly manifests cystic lung lesions
on HRCT.
92. NSIP EITHER
Idiopathic iNSIP OR Identifiable cause
• Connective tissue diseases
• Drugs
• Environmental/occupational exposures
• Immunocompromised hosts
• Infections
• Resolving acute lung injury
95. LIP
•idiopathic LIP
•Identifiable cause or underlying disease
Connective tissue disorders – esp. Sjögren
Immunodeficiency
Infections
Drugs/toxins
-Radiologically with GGO ,Cysts
96. Q 19
• A 50-year-old man, current smoker and HIV with CD4 500, has been complaining
of shortness of breath and non-productive cough for 5 months. He is previously
treated with antibiotics but his symptoms have failed to improve. In the
emergency department, he is noted to be hypoxic on room air and crackles on
auscultation of his lungs. His WBC 16,000; Hgb 14; Plt 300; LDH 500.He had a chest CT
which showed below
• The cell count from the bronchial alveolar lavage reveals eosinophils 5%,
lymphocytes 15%, neutrophils 15%.The transbronchial biopsy shows
inflammatory intraluminal plugs consisting of granulation tissue with fibroblasts
and myofibroblasts in connective matrix, in small airway, ducts and alveoli with
mild interstitial inflammation.There is preservation of architecture and uniform
appearance. What is your presumptive diagnosis?
a. Chronic eosinophilic pneumonia
b. Cryptogenic organizing pneumonia
c. Desquamative interstitial pneumonia
d. Pulmonary Oedema
e. Acute eosinophilic pneumonia
98. Q 20.
The patient has been discharged on prednisone 20 mg PO daily for 4 weeks.
He has not been compliance to his medications and he comes in
complaining of fatigue and shortness of breath. He is noted to have oxygen
saturation of 95% on room air and afebrile. Repeat radiographs show new
central sparing infiltrates on the left lung.Cultures have been obtained
which has been negative. What would be the next appropriate step?
a. Prednisone 20 mg PO daily
b. Solumedrol 1 g IV daily for 3 days
c. Piperacillin–Tazobactam 3.375 mg IV every 6 hours with vancomycin 1 g
IV every 12 hours
d. Cellcept 1,000 mg PO every 12 hours
e. Amphotericin B
99. A.
• Answer: A. Prednisone 20 mg PO daily
• The patient appears to have a relapse, which manifests as worsening
symptoms with reoccurrence of prior or new infiltrates.They are
common during steroid taper.
• Predictors of relapse include delayed treatment and mild increases
with alkaline phosphatase and gammaglutamyltransferase.
• Proposed taper of medications include prednisone 0.75 mg/kg/day for 4
weeks; followed by 0.5 mg/kg/day for 4 weeks, and then 20 mg daily
for 4 weeks, 10 mg for 6 weeks, and 5 mg daily for 6 weeks. If relapse
occur while dose <20 mg daily, increase dose to 20 mg daily and slowly
taper accordingly.
• Treatment of COP includes steroids from 0.75 to 1.5 mg/kg/day with
usual duration of up to 1 year.
100. Q 21.
• 40 y old female , progressive dyspnea for 5 m
• Abnormal CT Fig 1
• TBB reveals adenocarcinoma received cisplatin and gemcitabine EGFR
mutation positive , so started gefitinib . Her symptoms improve
• CT repeated after 2 m of treatment with no new symptoms except sob
Fig 2
• Bronchoscopy done and reveals lymphocytes 30 % CD4/CD8 3/3
• TBB Fig 3 ,Based on results what do you suggest ?
a. Begin Ganciclovir
b. Start radiation
c. Start Antibiotics
d. Stop gefitinib
103. Q 22
• 40 y old female , progressive dyspnea for 5 m
• Abnormal CT Fig 1
• TBB reveals adenocarcinoma received cisplatin and gemcitabine EGFR
mutation positive , so started gefitinib . Her symptoms improve
• CT repeated after 2 m of treatment Fig 2
• Bronchoscopy done and reveals lymphocytes 30 % CD4/CD8 3/3
• TBB Fig 3 ,Based on results what do you suggest ?
a. Begin Ganciclovir
b. Start radiation
c. Start Antibiotics
d. Stop gefitinib
104. Q.
• 25 y old lady not known to have
any medical illness , history of
recurrent abortions, presented
with dyspnea , admitted to ICU
CT shown - Bronchoscopy done
Which is true about this
condition?
a. Good prognosis
b. Bronchoscopy will not help in
diagnosis
c. Need high dose steroids
d. Complements will be normal
105. DAD
• Histologic pattern of ALI characterized by diffuse involvement with
edema, hyaline membranes, and acute interstitial inflammation
(exudative phase) evolving to loose organizing fibrosis and type II
pneumocyte hyperplasia (organizing phase).
• HRCT: Diffuse ground-glass and/or consolidative opacities
• Management: depends on the clinical context, corticosteroids
commonly used when non-infectious
• Prognosis: high short-term mortality
106. Acute Interstitial Pneumonia
Idiopathic (“Hamman Rich syndrome”)
Identifiable cause or underlying disease:
• Infections
•Toxic inhalants
• Drugs
• CTDs/vasculitides/alveolar hemorrhage
• Acute radiation reaction
• Acute exacerbation in ILDs
Histo – Septal thickening
and proliferation of spindle cells
107. Q 23
• 40 y old female teacher , Hypothyroidism and hypoadrenalism on
treatment, presented with shortness of breath ,cough a typical chest
pain and haemoptysis- minimal amount. History of Raynaud's and
generalized fatigability .No fever . No other systemic symptoms
• Looks Sick , Fully Oriented , BP 90/60 P130 Afebrile Spo2 90 %
• CVS S1, loud S2 with pansystolic murmur , Chest bilateral crackle and
L L oedema
• Leukopenia ,Mild elevation of transaminase
• CXR show Cardiomegaly and bilateral lung infiltrate
• Previous CT one y back :Interstitial lower lobes infiltrates with traction
bronchiectasis
• Echo Severe Pulmonary HTN 90 and dilated RA ,RV normal LV
• RHC ,ANA Positive ,all other autoimmune profile were negative
108. Q 23.
What is suggested treatment ?
A. Sildenafil ,Bosentan and Pirfenidone
B. illoprost , Bosentan and steroids
C. Lasix , Steroids ,Sildenafil and illoprost
D. Bosentan , illoprost ,Lasix and Steroids
E. Steroids only
109. Autoimmune-features ILD
(Interstitial Pneumonia with
Autoimmune Features (IPAF) )
.
Classification criteria:
• Presence of an interstitial pneumonia (by HRCT or SLBx) •
Exclusion of alternative etiologies and,
• Does not meet criteria of a defined connective tissue and, disease
and,
• At least one feature from at least 2/3 domains: clinical (e.g.,
Raynaud’s), serologic, morphologic (HRCT or SLBx features)
ERS/ATS statement. ERJ 2015
111. PPFE
• Idiopathic PPFE OR with Chemotherapy • Hematopoietic stem cell
transplant • Recurrent infections • Familial interstitial pneumonia
• Pleural and subpleural fibrosis with septal elastosis, predominantly
upper lobes
• HRCT: bilateral irregular pleuroparenchymal thickening, more
marked in upper and middle
• Spontaneous pneumomediastinum or pneumothorax common
• no effective treatment identified
• generally poor, most progress
112. Q. 24
Which is true about Familial Interstitial Pneumonia/ Familial Pulmonary
Fibrosis?
a. >20 % relatives
b. ~40% of interstitial pneumonia / pulmonary fibrosis
c. ~25% of these familial cases have identifiable mutations
d. Spescific HRCT and histopathologic pattern
e. None of the above
113. Familial Interstitial Pneumonia/
Familial Pulmonary Fibrosis
Evolving recommendations regarding
genetic testing for those with early onset
(<50 yr), positive family history, suspicious
extrapulmonary features
Borie et al. Eur Respir Rev 2017
114. Q 25
Which one of the following interstitial lung diseases is related to smoking?
A. LAM
B. Desquamative interstitial pneumonia
C. UIP
D. NSIP
E. DAD
115. Q
Which one of the following interstitial lung diseases is
NOT related to smoking?
A. Acute eosinophilic pneumonia
B. Desquamative interstitial pneumonia
C. Respiratory bronchiolitis –ILD
D. Hypersensitivity Pneumonitis
E. IPF
117. Q. 26
• 40 y old ,nurse ,15y pack history
• Progressive SOB
• No fever or haemoptysis
• RR 27 SPO2 93%RA
• Chest bilateral crepitation
• Normal Labs including ESR & ANA
• CT Shown
• Best treatment
a. Observation & Stop Smoking
b. Stop Smoking & Steriods
c. Steriods and azathioprin
d. Perfinedone
118. RB-ILD
• numerous pigmented macrophages
• Relatively uniform appearance
• Most are smoking-related
• HRCT: GGOs ± reticular opacities;
sometimes cysts, or and vague
nodules
• Management: smoking cessation,
corticosteroids
• Prognosis: generally good, up to 30%
mortality
119. Q 27
• 44 y old came with dyspnea on exertion and cough. Has been told
she has emphysema. Attempt of tobacco cessation failed.
• Physical examination reveals crackles
• Her radiographic ,pathology shown
• Which of the following is most likely ?
a. COPD
b. Goodpasture Syndrome with diffuse pulmonary haemorrhage
c. Pulmonary Langerhans histiocytosis
d. DIP
122. Combined Pulmonary Fibrosis and
Emphysema (CPFE)
•Upper lung emphysema and lower lung fibrosis
•Typically male smokers
•Relatively preserved spirometry and lung volumes with
low DLCO
•Increased incidence of pulmonary hypertension –
associated with increased mortality
•“Pulmonary fibrosis” includes UIP, NSIP, RB-ILD, DIP,
etc.
126. Q 28
Respiratory manifestations in CTD is characterized by
which one of the following?
A. Obstructive lung disease is not seen.
B. Lung biopsy is usually needed.
C. SLE involves the lung more often than other CTDs.
D. Acute exacerbation can occur in patients with CTD-
ILDs.
127. Rheumatic Disease ILD
•RA 20 - 30 %
•PM/DM 20 - 50 %
More common with anti-synthetase antibodies
•Systemic sclerosis 45 % (“clinically significant”)
More common in diffuse disease; topoisomerase-1
antibodies
•SLE 2 - 8 %
Usually in patients with multisystem disease
•MCTD 20 – 60 %
•Sjögren to 25 %
(Castelino et al. Arthritis ResTher 2010)
128. Q 29
45 year old man known case of PM/DM , presented with three
weeks history of dyspnea on exertion ,progressive and
associated with dry cough ,weight loss and loss of appetite He
has a history of Raynaud’s. Physical Examination show ankle
joint swelling, HRCT show bilateral interstitial infiltrate diffuse
predominantly upper lobes with traction bronchiectasis ILD.
What is true about this disease?
a. 2-5 % of PMDM patients will have it
b. UIP pattern is the commonest to be found in HRCT
c. Anti –jo antibodies positive
d. Obstructive ventilatory defect in PFT
e. None of the above
129. AntiSythestase Syndrome
•Subset (16-30%) of patients with PM/DM
• Characterized by relatively acute onset, constitutional
symptoms, Raynaud’s, “mechanic’s hands”, arthritis,
ILD.
•anti-Jo-1 (anti-histidyl–tRNA synthetase)
• Associated with ↑ risk of ILD Usually NSIP > UIP > OP;
sometimes LIP, DAD, etc
•Can be more refractory to treat than other PM/DM-
associated ILD
130. Q 30
• 30 y lady with SLE , Co progressive SOB 3 m , no other respiratory
symptoms . OnWarfarin for previous DVT. Examination is normal
• PFT FEV1 55% ,FVC 58% FEV1FVC 0.78 TLC 68%
RV 100%
DLCO 77% adjusted to alveolar volume 100%
• CXR small lung volume without lung infiltrates or effusion
What is the next to do ?
a. Echo
b. VQ scan
c. Maximum Inspiratory and Expiratory Pressure
d. Bronchoscopy
131. A.
• MIP ,MEP
• To assess muscle weakness ?myositis or phrenic n palsy
• Dx shrinking lung syndrome
• Tx steroid , theophylline , beta agonist and immunosuppression
132. Q 31
• 66 y old lady with Systemic sclerosis , Raynaud's
• Never smoker , work as a secretary
• Examination reveals Spo2 93% , diffuse skin thickening and
telangactasia upper limb digitals
• FVC 60%
• HRCT bilateral basal ground glass opacities
What is the best treatment ?
a. Cyclophosamide
b. Cyclosporine
c. Steroids
d. Azathioprine
133. A.
• B.
• RCT
• 49% improved with cyclophosphamide Vs 26%
Clin Rheumatol. 2006 Mar;25(2):205-12. Epub 2005 Oct 14.
A randomized unblinded trial of cyclophosphamide versus azathioprine in
the treatment of systemic sclerosis.
Nadashkevich O1, Davis P, Fritzler M, Kovalenko W
134. What is the Diagnosis?
LAM
Bronchiectasis
E
EG
135. Diffuse Cystic Lung Disease
• Cyst = a round parenchymal lucency with a well defined
thin-wall (<2 mm), usually contain air • Focal/multifocal
vs diffuse
•Cavity = a lucency within pulmonary consolidation, a
mass, or a nodule. (thick wall)
•Emphysema = focal areas of low attenuation without
visible walls
Fleischner Society, 2008
136. Mechanism of Cyst Formation
•Elastolysis mediated by matrix metalloproteinases
MMPs) – LAM, PLCH
• Destruction of the bronchial wall and progressive
luminal dilatation – PLCH
•Airway narrowing and check valve mechanism – LIP,
amyloidosis
• Hamartoma-like cystic alveolar formation – BHD
• Cavitation of nodule (inflammatory/infectious,
neoplastic)
137. Q 32
Which of the following radiographic features is
least likely to be found in Langerhans’ cell
histiocytosis of the lung?
a.Nodules ranging in size up to 10 mm
b.Bilateral reticulonodular opacities
c.Pneumothorax
d.Pleural effusion
e.Honeycomb lung
138.
139. Langerhans’ cell histiocytosis
(LCH)
d. Pleural effusion
1. Early , Centrilobular nodules (2–10 mm in size) and
2. Reticular and nodular opacities with a predominantly bilateral
symmetric upper- to mid-lung distribution.
3. Late ,Cysts develop and become the dominant imaging finding.
Cysts vary in size but usually are smaller than 1 cm may result
in bullous formation, which then predisposes the patient to
recurrent spontaneous pneumothorax. In advanced LCH,
honeycomb changes can occur.
4. Pleural effusions are rare.
Zaveri et al. 2014
141. Q 33
• 56 y F , smoker
• Secretary
• Progressive SOB for last 2 y
• Childhood asthma with FH of asthma
• Not on any medications
• Chest examination reduced breath
sounds -No skin lesions
• Previous- 1 y - CXR reported as
increase lung volume.
• FVC 79% FEV1 46% DLCO 60
What is the most likely diagnosis?
a.LAM
b.PLCH
c. Birt-Hogg-Dude syndrome
d.LIP
142. LAM
• Proliferation of abnormal
smooth muscle cells (LAM cells
– HMB-45+)
• Sporadic andTSC-related
forms; caused by mutation in
theTSC genes
• Mostly women
• High risk of pneumothorax: 60-
80%
• PFT:Typically obstructive
143. Diagnosis and Management -
LAM
CT chest findings plus any one or more of the following:
• Biopsy of lung or extrapulmonary LAM
• Renal angiomyolipomas
• Chylothorax (seen in 20-40% during course)
•Tuberous sclerosis complex (TSC)
• High serumVEGF-D level, >800 pg/mL Management
144. What is the name of this radiological
findings?
145. PAP
• Most are 20-60 y of age (median ~40)
• Nonspecific presentation: insidious onset DOE, cough - sometimes
asymptomatic
• Fever, fatigue, weight loss, chest pain, haemoptysis
• Inspiratory crackles in 20- 50%
• Serum LDH, surfactant A and D, KL-6 (mucinlike glycoprotein) -
common, nonspecific
• Anti-GM-CSF antibodies detectable in serum & BAL fluid in most
cases of acquired PAP
• PFTs: a restrictive defect, reduced DLCO
• Whole Lung Lavage
147. Radiation-induced Lung Injury
After radiation therapy in patients with lung cancer and mediastinal
lymphoma, radiologic abnormalities occur in 60-90%; 5-15%
symptomatic.
Radiation pneumonitis - symptoms 4-12 weeks after irradiation
Radiation fibrosis - 6-12 months after irradiation
• Imaging: radiographic abnormalities confined to radiation field
with “straight line” effect
• Management: symptomatic pneumonitis, prednisone 40-60 mg/d x
2 wk, then taper over 4-12 wk