Pulmonary Manifestations of Systemic Diseases
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This document summarizes various pulmonary manifestations of systemic diseases. It discusses pulmonary complications related to hematopoietic stem cell transplant, solid organ transplant, abdominal diseases, hematological disorders, endocrine disorders, and neuromuscular disorders. For each category, it provides brief descriptions of specific conditions and their associated risks, symptoms, diagnostic evaluations, treatments, and prognoses.
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Pulmonary Manifestations of Systemic Diseases
- 1. Pulmonary Manifestations of Systemic Diseases Dr.S.Sesha Sai (PG) Department of Pulmonary Medicine SMC,VJA.
- 2. ©drseshas Systemic Diseases • HSCT and solid organ transplant • Abdominal diseases • Hematological disorders • Endocrine disorders • Neuromuscular disorders • Chest wall abnormalities • CTD • Pregnancy
- 3. ©drseshas HSCT and Solid organ transplant
- 4. ©drseshas Hematopoietic Stem Cell Transplant Pre Engra f Early Post Transplant Late Post Transplant
- 5. ©drseshas Non Infectious Pulmonary Complications in HSCT recepient
- 6. ©drseshas Condition Pulmonary Function Upper Airway Complications Bronchiolitis Obliterans Briefing •PFT abnormalities without symptoms. Injury to mucosal barrier Small airway inflammation and fibroprolif process with airflow limitation Risk Factors Smoking, Old age, CT, GVHD, HLA mismatch TBIR, High dose steroids TBIR, Delayed engraftment, Leukemia GVHD, Age, Mtx use Clinical Findings Laryngeal edema, Dysphagia, Asp pneumonia Dry cough, Dyspnea, Exercise intolerance, Wheeze Fever - absent Diagnosis •Restr & Obst •Gas exchange abn •Respiratory muscle weakness(restr) FEV1/FVC < 0.7, FEV1<75% RV > 120% Exclude Infection – Lab , CT,BAL Radiology Air trap, Small airway thickening, Bronchiectasis Treatment Supportive, ETT Corticosteroids GVHD – azathioprine, tacrolimus, mycophenolate Macrolides – 12 wks Extra corporeal Chemotherapy – chronic GVHD Prognosis Poor, 5-YSR – 45%
- 7. ©drseshas Condition Peri - engraftment RDS (PERDS) Acute Pulmonary edema Idiopathic Pneumonia Syndrome Briefing Pulmonary manifestation of engraftment syndrome Engraftment syndrome – rash, pulm infiltrates, fever, diarrhea, capillary leak Lung injury without infectious etiology Criteria – Alveolar injury, Absence of LRTI, Exclusion of cardiac dysfunction, renal failure, fluid overload Risk Factors Stem cell dose & source, growth factor use, amphotericin TBIR, Induction drugs, Sepsis Pre transplant medications – CMV prophylaxis Clinical Findings Dyspnea, Fever Hypoxemia CXR pulmonary infiltrates c/n 5 days of neutrophilic engraftment Dyspnea Basilar crackles Cough, Dyspnea Hypoxemia Diagnosis Neutrophilic engraftment (ANC>500/day on consec days) Exclude inf - BAL CXR – ggo/consolidation, enlarged heart, small pleural effusions Non lobar infiltrates Histology - Inflammation without evidence of infection Radiology CXR – Pulm infiltrates CT - prominent vessels, GGO, Pl.E, Interlobular septa thickning Treatment Corticosteroids Fluid restriction Diuretics Supportive care Prevention and treatment of infection Steroids – no benefit Prognosis Good. 1/3rd - Intubation Poor 1YSR – 15%
- 8. ©drseshas Condition Diffuse Alveolar h’age Cryptogenic Organizing Pneumonia Briefing Small muscular artery vasculopathy and thrombotic micro angiopathy Organizing pneumonia in alveolar ducts and alveoli c/c out bronchiolar involvement Risk Factors Pre transplant CT, Age, TBIR GVHD Clinical Findings Dry cough, Dyspnea Dry cough, Dyspnea, fever Diagnosis BAL - > 20% hemosiderin laden macrophages, blood in at least 30% alveoli PFT- restrictive Lung biopsy Radiology B/L fine reticular opacities CXR & CT – GGO. Nodules, air space consolidation Treatment Systemic corticosteroids Corticosteroids Prognosis Poor Poor
- 9. ©drseshas Other Conditions • Delayed Pulmonary Toxicity Syndrome (DPTS) • Pulmonary Cytolytic Thrombi (PCT) • Pulmonary Veno occlusive disease • Pulmonary Alveolar Proteinosis • Chronic Eosinophilic Pneumonia • TRALI • Sarcoidosis Interstitial pneumonitis fibrosis, Corticosteroids No deaths Fever Pulmonary Nodules Antibiotics and Steroids No deaths
- 10. ©drseshas Solid Organ Transplant Peri operative Complications Pleural Effusions • Liver transplant – Disruption of lymphatics • Transudative • Right or Bilateral • Resolve by 3rd week Diaphragmatic disorders • Liver – Right dysfunction. Crush injury to right phrenic nerve by suprahepatic vena caval clamp • Heart • Dysfunction - right phrenic • Diaphragmatic Hernias Neoplastic disorders • Post Transplant Lympho proliferative Disorder (PTLD) • Immunosuppression • Pulmonary nodules, LN ,Pl Ef. • Reduce Immunosuppression • Rituximab • Bronchogenic Carcinoma – Heart transplant Drug Induced Lung Disease • Sirolimus • Interstitial Pneumonitis • Discontinue & Steroids • OKT3 & Basiliximab – Non cardiogenic Pulmonary Edema Pulmonary Metastatic Calcification • Liver Transplant • Sec Hyperparathyroidism • No treatment
- 11. ©drseshas Peri operative Complications • Liver ▫ Impaired Respiratory Muscle function due to extensive surgery ▫ Hepatopulmonary syndrome and Porto pulmonary hypertension ▫ HPS – Liver ds., arterial hypoxemia, intrapulmonary vascular dilatation • Heart – same risk as other cardiac surgeries • Kidney – Few perioperative complications
- 13. ©drseshas •Gastro esophageal and Intestinal diseases •Hepatic Diseases •Pancreatitis •Kidney Diseases
- 14. ©drseshas Gastro esophageal and Intestinal Diseases
- 15. ©drseshas GERD • Respiratory Complications - Cough, chronic bronchitis, pneumonia, bronchiectasis, idiopathic pulmonary fibrosis, stable COPD and COPD exacerbations, Bronchiolitis Obliterans syndrome after lung transplantation, and nontuberculous mycobacterial lung disease • Mechanism – microaspiration, activation of a vagal reflex • Causes ▫ Obstructive sleep apnea ▫ Hyperinflation and gas trapping may flatten the diaphragm ▫ Bronchodilator therapy ▫ Theophylline
- 16. ©drseshas • Symptoms ▫ heartburn, regurgitation, or Dysphagia. ▫ substernal chest pain, hoarseness, sore throat, otalgia, hiccups, or even tooth erosion. • Ambulatory reflux monitoring (pH or impedance- pH) • Treatment recommendations ▫ weight loss ▫ elevation of the head of the bed, ▫ avoidance of eating within 2 to 3 hours before bedtime. • PPI is the therapy of choice – 3 months • Prokinetic agents • Nissen fundoplication
- 17. ©drseshas Inflammatory Bowel Disease • Ulcerative Colitis > Crohn’s disease • Crohn’s – Colobronchial and Broncho esophageal fistula • Anti inflammatory drugs – Sulfasalazine, Azathioprine • Inhaled or Systemic corticosteroids.
- 19. ©drseshas Hepatic Diseases • Porto pulmonary Hypertension • Pleural Effusion • Pulmonary Function Disturbances – Decreased DLCO • Primary Biliary Cirrhosis • Chronic Active Hepatitis – Pulmonary Fibrosis, LIP • Sclerosing Cholangitis - Bronchiectasis • Alpha1-Antitrypsin Deficiency - COPD • Hepatopulmonary Syndrome Transudates Right Repeated thoracenteses- transitory effects Thoracostomy tube drainage - protein loss. Transjugular intrahepatic portosystemic shunting ILD – LIP and fibrosing alveolitis Intrapulmonary granulomas High CD4 in BAL Bronchiectasis
- 20. ©drseshas Hepatopulmonary Syndrome • Severe hypoxemia (arterial PO2 < 60 mm Hg) with uncomplicated chronic hepatic disease. • HPS triad: Advanced chronic liver disease, Arterial hypoxemia, widespread pulmonary vascular dilations. • Cyanotic, finger clubbing, shortness of breath and platypnea • Criteria for HPS are as follows: ▫ Liver disease, ▫ Increased A-a gradient (>15 mm Hg) ▫ With or without arterial hypoxemia ( < 80 mm Hg) ▫ Positive CE –ECHO or an abnormal intravenous radiolabeled perfusion lung scan • 2D CE – ECHO : Micro bubbles of air in the left heart cavities within 3 to 6 beats of their visualization in the right-sided chambers • Treatment – Cannot be corrected with Oxygen, Liver transplantation
- 21. ©drseshas
- 22. ©drseshas Pancreatitis Respiratory failure Pleural Effusion •ARDS •Toxic effect of Pancreatic Products •Left sided •Elevated Amylase in Pleural fluid •Exudate •Bloody (sometimes) •Chronic Effusions – Pancreaticopelural fistula •Supportive •Thoracocentesis •Surgery - fistula
- 23. ©drseshas Kidney Diseases Pulmonary Edema • Dialysis Pleural Disease • Pleuritic pain • Effusion • Pleural rub • Fibrothorax • Surgicl Decortication Pulmonary Calcification • Metastatic calcification Sleep Apnea • Fluid shift to upper body Dialysis induced Hypoxemia • O2 supplementation
- 24. ©drseshas Hematological Diseases Most of the hemolytic disorders lead to PH
- 25. ©drseshas RED BLOOD CELL DISORDERS Anemia - Low o2 capacity, PH Polycythemia - Pulmonary Nodules, Pleuropulmonary masses (haematopoiesis) HEMOGLOBINOPATHIES Sickle Cell Disease Thalassemia • Complications of therapy – transfusion related • Pulmonary parenchymal masses, mediastinal masses, pleural effusions • PH
- 26. ©drseshas WBC DISORDERS Leukemias Plasma Cell Disorders • Left pleural effusion • Pulmonary parenchymal or mediastinal or chest wall plasmocytomas THROMBOSIS AND DISORDERS OF COAGULATION Pulmonary hemorrhage, Hemomediastinum, tracheal and pleural hematomas Mediastinal and lymph node involvement Pleural and pulmonary infltration Leukemic cell lysis pneumopathy -- Tumor lysis syndrome Hypoxia and inflitates within 48hr of chemotherapy Aggressive intravenous hydration, urine alkalinization Allopurinol and rasburicase
- 27. ©drseshas COMPLICATIONS OF TRANSFUSION • Transfusion-Associated Acute Lung Injury (TRALI) • Whole blood • Acute onset of hypoxemia (PO2/FIO2 < 300 mm Hg), New chest radiographic opacities within 6 hours of infusion of a blood product • Transfused leukocyte antigens interact with antibodies - granulocyte activation and lung injury. • Symptoms - Sudden onset of respiratory distress 1 to 2 hours after the transfusion of blood products. • Clinical signs include fever, tachypnea, tachycardia, and occasionally hypotension. Pink frothy secretions with a high albumin content (NCPE) • Chest radiography - bilateral alveolar opacities • 70% - mechanical ventilation at the time of presentation. • The illness tends to resolve rapidly, within the frst 48 hours.
- 28. ©drseshas Sickle Cell Disease • Acute ▫ Asthma ▫ Acute Chest Syndrome • Chronic ▫ Pulmonary Fibrosis ▫ PH and Cor pulmonale
- 29. ©drseshas Acute Chest Syndrome • Increased membrane permeability that characterizes the acute respiratory distress syndrome (ARDS). • New pulmonary opacity (consolidation) – one segment, consolidation • Fever, cough, chest pain, tachypnea, dyspnea, and abdominal, arm, leg, rib, or sternal pain • Causes ▫ Infection (chlamydia, mycoplasma, RSV) ▫ Bone marrow fat embolization (infarction & necrosis of bone) ▫ Intravascular sequestration causing lung injury and infarction
- 30. ©drseshas • Diagnosis - Oil Red O-positive lipid accumulations within alveolar macrophages in Sputum or BAL. • Treatment ▫ O2 therapy ▫ Pain control ▫ Asthma therapy ▫ Antibiotics ▫ Transfusion – Simple transfusion is as effective as erythrocytapharesis
- 32. ©drseshas • Diabetes mellitus • Thyroid disorders ▫ Hyperthyroidism  Asthma may worsen if β – blockers given ▫ Hypothyroidism  Dyspnea on exertion  Obstructive and central sleep apnea  Respiratory muscle and diaphragmatic muscle weakness  Effusions – trans/exudate  Thyroid replacement therapy
- 33. ©drseshas • Parathyroid diseases ▫ Metastatic calcification - apices (ESRD) ▫ PH • Adrenal diseases ▫ Hypercortisolism - mucocutaneous fungal and opportunistic pulmonary infections ▫ Insufficiency – VLBW infants - BPD • Acromegaly ▫ Macroglossia, nasal polyps, oropharyngeal airway narrowing ▫ Sleep apnea, extrathoracic airway obstruction, vocal cord dysfunction ▫ Diffcult intubation ▫ Pituitary ablation or somatostatin analogues
- 35. ©drseshas
- 36. ©drseshas • Ondine’s Curse • Spinal cord ▫ Above C3 – ventilatory support ▫ C3 to C5 – varies ▫ Below C5 – independent ▫ Sleep Apnea
- 37. ©drseshas
- 38. ©drseshas • Motor disorders – respiratory and diaphragm muscle weakness • Loss of cough – upper respiratory muscle • May require NIV. • Myopathy – Muscle wasting, weakness
- 39. ©drseshas Special Conditions • Critical illness polyneuromyopathy / ICU – related weakness ▫ Etiology – not known ▫ Intensive insulin therapy (80 – 110 mg/dl) • Diaphragm dysfunction ▫ Unilateral  Elevated Hemidiaphragm  Sniff test – Paradoxical movement fluoroscopy or USG.  No specific treatment  50% recovery
- 40. ©drseshas ▫Bilateral MC – Amyotropic Lateral Sclerosis Phrenic nerve conduction studies – cause Transdiaphragmatic pressure using thin balloon-tipped polyethylene catheters placed in the esophagus and stomach No specific treatment
- 41. ©drseshas
- 42. ©drseshas Management of Neuromuscular Disorder • Cough Support ▫ Manual ▫ Frog Breathing techniques  Breath stacking  Glossopharyngeal Breathing ▫ Mechanical Insufflator – Exsufflator/ Cough Assist
- 45. ©drseshas Pectus Excavatum • Excessive depression of the sternum and its adjacent costal cartilages • Tr/AP ratio, also known as the Haller index, is 2.5 or less normally. • A Haller index of greater than 3.25 - signifcant pectus deformity that may need surgical correction • Surgical correction Flail Chest • Hypoventilation due to pain, flail-induced impairment in respiratory muscle function, concomitant lung injury • Adequate analgesia, clearing of bronchial secretions • Positive pressure ventilation
- 46. ©drseshas Ankylosing Spondylitis • Inflammation - Ligamentous structures of the spine, the sacroiliac joint, and large peripheral joints limiting rib cage motion. • Stiffening and fusion of the costovertebral and sternoclavicular articulations • Restrictive lung disease • Fibrobullous upper lobe disease • Caution in intubation hyperextension of a rigid cervical spine may lead to fracture • AntiTNF-α therapy Obesity • Simple obesity individuals who are eucapnic • OHS, individuals who retain CO2) • Weight loss, bariatric surgery • Arterial PO2 roughly improves by 1 mm Hg for every5 kg reduction in weight
- 47. ©drseshas Kyphoscoliosis • Idiopathic, secondary or paralytic, congenital. • Adam forward bend test • Cobb angle (higher = severe deformity) ▫ >100 degrees - dyspnea on exertion ▫ >120 degrees - respiratory failure • Most severe restrictive impairment of all chest wall diseases • Sleep disorders, Hypoventilation, Hypoxia, Hypercapnia
- 48. ©drseshas • Idiopathic – benign course, Secondary – rapid course • Treatment ▫ Immunizations ▫ Smoking cessation ▫ Maintenance of a normal body weight ▫ Supplemental oxygen ▫ Prompt treatment of respiratory infections ▫ Orthopedic braces ▫ Non invasive Nocturnal Ventilation
- 49. ©drseshas References • Fishmans Pulmonary Diseases and Disorders 5th edition • Murray and Nadels Textbook of Respiratory Medicine 6th edition
- 50. ©drseshas
Editor's Notes
- Lymphoma, leukemia new blood-forming cells start to grow and make healthy blood stem cells
- Osa bronchospasm and cough are accompanied by an increase in the negative pressure within the thorax, and hence in the esophagus, hyperinflation, allowing the lower esophageal sphincter to be drawn up Bronchodilators increases gastric acid secretion and decreases lower oesophageal sphincter tone
- improve esophageal contractility and increase both lower esophageal sphincter pressure and gastric emptying.