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  • 1. Case Presentations By Gamal Rabie Agmy , MD , FCCP Professor of Chest Diseases ,Assiut University
  • 2. Third Session SIX CASES
  • 3. Case No. 1
  • 4. • A 62-year-old woman who has idiopathic pulmonary fibrosis (IPF) has rapidly worsening dyspnea over the past 3 weeks. • She was diagnosed with IPF by CT scan and surgical lung biopsy 1 year ago. • At that time, she started to receive prednisone and cyclophosphamide and had been fairly stable until these past few weeks
  • 5. • On physical examination: She is in mild respiratory distress. Her BP is 118/68 mm Hg, Temperature is 37.6C, Heart rate is 115/min, Respiratory rate is 22/min, and oxygen saturation is 90% on 4 L/min supplemental oxygen, increased from a baseline of 2 L/min.
  • 6. • She has no jugular venous distention or neck adenopathy. • She is tachycardic without any extra heart sounds or murmurs. • Lung examination reveals bibasilar crackles without any signs of focal consolidation. • There was no peripheral edema.
  • 7. • Her chest radiograph reveals diffuse patchy opacities and bibasilar reticular markings. • Her CBC is remarkable for a WBC count of 8,700/L (8.7 × 109/L), with 60% neutrophils, 1% bands, 20% lymphocytes, 18% monocytes and 1% eosinophils. • Brain natriuretic peptide level is 100 pg/mL (100 ng/L)
  • 8. • Serial troponin T levels are not elevated. • Blood, sputum, and urine culture results are negative. • CT pulmonary angiogram does not reveal any evidence of pulmonary embolism.
  • 9. Which of the following statements about her diagnosis is correct? A. The CT angiogram will demonstrate progression of interstitial disease and increased cyst formation. B. Bronchoalveolar lavage will reveal an eosinophilic cellular infiltrate. C. Lung biopsy will show changes consistent with diffuse alveolar damage. D. Most patients will have a favorable response to pulse treatment with corticosteroids
  • 10. • This patient meets the diagnostic criteria for an acute exacerbation of IPF (AE-IPF): Worsening of dyspnea within the past month, Increased hypoxemia, New pulmonary opacities on chest radiography, The absence of apparent infection or heart disease. • AE-IPF is an acute insult to the lung with a background of IPF, and the pathologic changes reflect this fact.
  • 11. • The histopathologic findings reveal hyaline membranes lining the alveoli, type II pneumonocyte proliferation, and interstitial inflammation and fibrosis, ie, diffuse alveolar damage over and above the underlying usual interstitial pneumonia (choice C is correct)
  • 12. • IPF is often thought of as a slow, but inevitable decline in pulmonary function. • However, newer data suggest that, in many patients, the disease is characterized by only minimal physiologic deterioration but frequent hospitalizations for respiratory disorders and an acute and rapid progression of lung disease in almost half the patients who die from IPF. • The incidence of this acute decline in all patients with IPF appears to be around 10% to 15%/year
  • 13. • As noted above, AE-IPF is defined as rapid deterioration during the course of the disease that is not due to infection, pulmonary embolism, or heart disease. • It is crucial to rule out these latter possibilities, as treatment for one condition could be fatal for the other. • Rapidly progressive dyspnea is the most common symptom, although patients may also complain of a nonproductive cough and low-grade fevers. No clear risk factors have been identified, and there is often no identifiable trigger
  • 14. • The pathogenesis is not clear. Evidence suggests that IPF involves multiple microscopic injuries to the lungs that are temporally distributed over many years. These injuries lead to inflammation stimulated by an imbalance in T-helper type II cells and subsequent cytokine release and fibroblast proliferation. • These injuries likely account for the temporal heterogeneity seen in UIP and may also be responsible for more acute episodes characterized by a rapid decline in lung function.
  • 15. • Chest radiographs will reveal new opacities, and CT scans will show new-onset diffuse ground-glass opacities (GGO) and/or consolidation on the background of the existing fi brosis and honeycombing (HC) (choice A is incorrect). • Bronchoscopy with BAL is often done to exclude infection and will typically show BAL neutrophilia, large numbers of alveolar macrophages, and desquamated alveolar cells with varying degrees of nuclear atypia (choice B is incorrect).
  • 16. • There are no large trials on treatment. Most patients are given a pulse of methylprednisolone and their usual steroid dose is increased. If they are not already receiving another immunosuppressive agent, one may be added. • Unfortunately, reported patient series show that a large percentage of patients will go on to require mechanical ventilatory support, and the majority of these patients will die of respiratory failure (choice D is incorrect).
  • 17. SO which of the following statements about her diagnosis is correct? A. The CT angiogram will demonstrate progression of interstitial disease and increased cyst formation. B. Bronchoalveolar lavage will reveal an eosinophilic cellular infi ltrate. C. Lung biopsy will show changes consistent with diff use alveolar damage. D. Most patients will have a favorable response to pulse treatment with corticosteroids
  • 18. Acute exacerbations in patients with idiopathic pulmonary fibrosis By Gamal Rabie Agmy , MD , FCCP Professor of Chest Diseases ,Assiut University
  • 19. Histopathological Patterns of IIPs Thannickal VJ, et al. Annu Rev Med. 2004;55:395-417. Age Genetic factors Environmental factors Nature of injury – Etiologic agent – Recurrent vs single – Endothelial vs epithelial Histopathologic Pattern DIP RB-ILD LIP COP NSIP AIP UIP Inflammation Fibrosis LUNG INJURY
  • 20. 50% Years Respiratory Function/Symptoms 1 2 3 4 FVC Traditional View of UIP/IPF Progression Progression of IPF: Acute Exacerbation vs Slow Decline FVC = forced vital capacity
  • 21. 50% Years Respiratory Function/Symptoms 1 2 3 Acute exacerbation Step Theory of UIP/IPF Progression Progression of IPF: Acute Exacerbation vs Slow Decline FVC 0 4 Am J Respir Cell Mol Biol. 2003;29(3 suppl):S1-S105. =hits
  • 22. Multiple Hypotheses for the Pathogenesis of IPF • Inflammation causes fibrosis • Noninflammatory (multiple hit) hypothesis: fibrosis results from epithelial injury and abnormal wound healing in the absence of chronic inflammation • Vascular remodeling: aberrant vascular remodeling supports fibrosis, and may contribute to increased shunt and hypoxemia Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii. Raghu G, Chang J. Clin Chest Med. 2004;25: 621-636, v. Strieter R. Am J Respir Cell Mol Biol. 2003;29(3 suppl):S67-S69.
  • 23. • Inflammation causes fibrosis – Inflammatory concept was dominant in the 1970s and 1980s • IPF resulted from unremitting inflammatory response to injury culminating in progressive fibrosis – Role of inflammation remains controversial • Lack of efficacy of corticosteroids Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii. Raghu G, Chang J. Clin Chest Med. 2004;25:621-636, v. Injury Inflammation Fibrosis Inflammatory Hypothesis
  • 24. Injury Epithelial cells Slide courtesy of Paul Noble, MD. Progression of Lung Fibrosis Capillary Endothelial cells ?
  • 25. Epithelial cells Collagen Myofibroblast Cell death Growth factors and other products of epithelial cell Injury Slide courtesy of Paul Noble, MD. Tissue Model of Lung Fibrosis Capillary Endothelial cells
  • 26. • Fibrosis results from epithelial/endothelial injury and abnormal wound healing in the absence of chronic inflammation – Recurrent, unknown injury to distal pulmonary parenchyma causes repeated epithelial cell injury and apoptosis – Loss of alveolar epithelium exposes basement membrane to oxidative injury and degradation – Failure of re-epithelialization/re-endothelialization provides stimulus for persistent profibrotic growth factor production, persistent fibroblast proliferation, excessive deposition of ECM, and progressive fibrosis Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii. Raghu G, Chang J. Clin Chest Med. 2004;25:621-636, v. Selman M, et al. Drugs. 2004;64:405-430. Noninflammatory (multiple hit) Hypothesis
  • 27. Noninflammatory (multiple hit) Hypothesis Recurrent pulmonary injury Epithelial/ endothelial injury and apoptosis Loss of basement membrane Failure of re-epithelialization/ re-endothelialization ECM deposition Fibroblast proliferation Release of profibrotic growth factors (TGF-b, PDGF, IGF-1) Progressive fibrosis with loss of lung architecture TGF-b = transforming growth factor-beta PDGF = platelet derived growth factor IGF-1 = insulin-like growth factor-1 Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii. Raghu G, Chang J. Clin Chest Med. 2004;25:621-636, v. Selman M, et al. Drugs. 2004;64:405-430.
  • 28. • Aberrant vascular remodeling supports fibrosis and may contribute to increased shunt and hypoxemia  Increased angiogenesis results from imbalance of pro-angiogenic chemokines (IL-8, ENA-78) and anti-angiogenic, IFN-inducible chemokines (IP-10)  Vascular remodeling leads to anastomoses between the systemic/pulmonary microvasculature, increasing right-to-left shunt, contributing to hypoxemia Chemokine imbalance Increased angiogenesis Fibrosis Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii. Strieter RM, et al. Am J Respir Cell Mol Biol. 2003;29(3 suppl):S67-S69. Vascular Remodeling Hypothesis Aberrant vascular remodeling
  • 29. Defects in Host Defense Mechanisms May Contribute to Fibrosis • Defects in endogenous host defense mechanisms (eg, IFN-g, PGE2 production) that limit fibrosis after acute lung injury may contribute to progressive fibrosis Noble PW, Homer RJ. Clin Chest Med. 2004;25:749-758, vii.
  • 30. NHLBI in an attempt to standardize the diagnostic criteria used across studies . This committee defined AEx-IPF as an acute, clinically significant deterioration of unidentifiable cause and proposed five diagnostic criteria. Definition of AEx-IPF
  • 31. 1- Previous or concurrent diagnosis of IPF 2- Unexplained worsening or development of dyspnea within 30 days 3- HRCT with new bilateral ground-glass abnormality and/or consolidation superimposed on a background reticular or honeycomb pattern consistent with UIP pattern 4- No evidence of pulmonary infection by endotracheal aspirate or BAL 5- Exclusion of alternative causes, including: • Left heart failure • Pulmonary embolism • Identifiable cause of acute lung injury. Diagnostic criteria for AEx-IPF
  • 32. Incidence of AEx-IPF *American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) on the diagnosis and treatment of IPF state that AEx- IPF occurs in approximately 5–10% of patients with diagnosed IPF annually *A recent retrospective study of data collected from 461 patients with diagnosed IPF found 1-year and 3- year incidences of AEx-IPF of 14.2% and 20.7%, respectively . *However, the incidence rates of AEx-IPF reported in clinical trials have tended to be lower than this.
  • 33. Pathophysiology of AEx-IPF A variety of patterns of acute lung injury have been observed in AEx-IPF . The most common histopathological finding is diffuse alveolar damage superimposed on the underlying usual interstitial pneumonia (UIP) pattern , but organizing pneumonia and extensive fibroblastic foci have also been reported. Several hypotheses for the etiology of AEx-IPF have been proposed. AEx-IPF may represent a sudden acceleration of the underlying disease process due to unknown acute injury to the lung, or a biologically distinct pathological process due to a clinically occult condition, such as infection or gastroesophageal reflux disease (GERD)
  • 34. Pathophysiology of AEx-IPF As AEx-IPF have a clinical presentation that shares a number of features with viral respiratory infections (e.g. fever, cough, myalgia), it has been suggested that occult viral infection may contribute to the pathophysiology of AEx-IPF. Several hypotheses for the etiology of AEx-IPF have been proposed. AEx-IPF may represent a sudden acceleration of the underlying disease process due to unknown acute injury to the lung, or a biologically distinct pathological process due to a clinically occult condition, such as infection or gastroesophageal reflux disease (GERD). However, the evidence supporting the involvement of viral infections in AEx- IPF is mixed .The most recent and extensive study, which used genomic-based technologies to investigate the role of viruses in the etiology of AEx-IPF, suggested that viral infection is not a common cause ofAEx-IPF.
  • 35. Pathophysiology of AEx-IPF Activation of the immune system, disordered coagulation/fibrinolysis, and oxidative stress may all contribute to the pathophysiology of AEx-IPF. Immune cells (e.g. neutrophils, macrophages) ,inflammatory mediators (e.g. interleukin 6, high mobility group protein B1) ,markers of coagulation/fibrinolysis (e.g. protein C, thrombomodulin, and plasma activator inhibitor-1) , and markers of oxidative stress (thioredoxin 1) are all elevated in patients with AEx-IPF. Epithelial cell damage in patients with IPF is demonstrated by over-expression of matrix metalloproteinase (MMP)-7, MMP-9 , and Krebs von den Lungen- 6 (KL-6) . Accelerated epithelial cell proliferation, with increases in the proliferation markers CCNA2 and Ki-67, in patients with AEx-IPF may be a compensatory response to injury, and is associated with epithelial cell death. Transforming growth factor (TGF)- beta, a fibrogenic cytokine, is upregulated in IPF and galectin-3, a mediator of fibrosis induced by TGF-beta, is elevated in the lungs and serum of patients with stable IPF and AEx-IPF . Circulating bone marrowderived fibrocytes may also provide a source of lung fibroblasts and myofibroblasts, as the number of circulating fibrocytes has been shown to be higher in patients with IPF and AEx-IPF, compared with healthy subjects
  • 36. Risk factors and precipitating factors for AEx-IPF *Lower total lung capacity, lower forced vital capacity (FVC) and/or lower diffusing capacity of the lung for carbon monoxide (DLco). *A higher degree of dyspnea (score ≥2 on the modified Medical Research Council dyspnea scale) or of fibrosis on HRCT has been shown to increase the risk of AEx-IPF, as has the presence of concomitant conditions such as emphysema or pulmonary hypertension. *Invasive examinations such as bronchoscopy , bronchoalveolar lavage (BAL), and pulmonary resection for lung cancer can precipitate AEx-IPF.
  • 37. Risk factors and precipitating factors for AEx-IPF *surgical lung biopsy is a precipitating factor for AEx-IPF; however, the risk of AEx-IPF from video-assisted thoracoscopic operation appears to be elevated only in patients with severe physiologic impairment or substantial comorbidity *In some patients with AEx-IPF, pepsin levels were found to be elevated in BAL fluid, suggesting a possible role for GERD in the pathogenesis of AEx-IPF .There is some evidence to suggest that the treatment of GERD in patients with IPF reduces mortality rates .
  • 38. Impact of AEx-IPF on patients *AEx-IPF are certainly a leading cause of hospitalization and death among patients with IPF. Median survival after an AEx- IPF has been reported to be between 22 days and 4.2 months. *There is some evidence that patients with better lung function (FVC, PaO2, DLCO) prior to AEx-IPF are more likely to survive an AEx-IPF , suggesting that preservation of lung function may be an important way of reducing the impact of AEx-IPF in patients with IPF.
  • 39. Management of AEx-IPF *The latest international treatment guidelines state that supportive care remains the mainstay of treatment for AEx-IPF, but also give a weak recommendation for the treatment of the majority of patients with AEx-IPF with corticosteroids. *In clinical practice, the treatment of AEx-IPF is variable. Corticosteroids (e.g. prednisone, methylprednisolone) are used in the majority of patients who suffer an AEx-IPF, usually in pulse doses. Preliminary data suggest that response to high- dose corticosteroid treatment may depend on the type of HRCT lesion, with better responses achieved in those with a peripheral pattern
  • 40. Management of AEx-IPF *Broad-spectrum antibiotics and immunosuppressants (cyclosporin or cyclophosphamide) are sometimes used in addition to corticosteroids .However, the efficacy of immunosuppressants in the treatment of AEx-IPF is based on a few small retrospective studies that do not provide conclusive evidence for benefit. *Mechanical ventilation is often used in patients with AEx-IPF, but the data on its effects on outcomes are mixed. *Other treatments for AEx-IPF that havebeen investigated in small studies include polymyxin Bimmobilized fiber column (PMX) hemoperfusion and tacrolimus, a cytokine transcription inhibitor ,usually administered in addition to corticosteroids.
  • 41. Reducing the risk of exacerbations *A trial of sildenafil, a phosphodiesterase-5 inhibitor, showed a numerical reduction in AEx-IPF in patients given sildenafil versus placebo (3 [3.4%] vs. 7 [7.6%]), but the number of events was small and the difference was not statistically significant . *Imatinib, a tyrosine kinase inhibitor , bosentan, an endothelin receptor antagonist, the anticoagulant warfarin , and inhaled Nacetylcysteine , numerically higher rates of AEx-IPF were found in the active treatment arms compared with the placebo arms. *triple therapy with prednisone, azathioprine,and N- acetylcysteine in patients with IPF, a significantly higher rate of AEx-IPF was observed in patients receiving triple therapy versus placebo
  • 42. Reducing the risk of exacerbations *Pirfenidone, an anti-fibrotic molecule that has been licensed for the treatment of IPF in Japan, India, China, Europe, and Canada, but was not approved in the United States, has shown inconsistent effects on AEx-IPF. *Nintedanib (formerly known as BIBF 1120) is a tyrosine kinase inhibitor in clinical development for the treatment of IPF. It reported a lower incidence of AEx-IPF was observed in patients treated with nintedanib 300 mg/day than placebo (2.4 vs. 15.7 AEx-IPF per 100 patient years) *It is interesting that nintedanib may have an effect on AEx-IPF whereas the tyrosine kinase inhibitor imatinib, which inhibits the platelet-derived growth factor receptor (PDGFR), did not . Nintedanib is an inhibitor of PDGFR, vascular endothelial growth factor receptor (VEGFR), and fibroblast growth factor receptor (FGFR) and this specificity of inhibition may be key to its effects on AEx-IPF
  • 43. Reducing the risk of exacerbations *Anti-acid treatment might decrease the frequency of AEx-IPF by reducing the acidity of the microaspirate .
  • 44. Case No. 2
  • 45. • A 46-year-old man is seen because of 4 months of increasing cough. • He has HIV infection, his CD4 lymphocyte count 3 months ago was 240/L (0.24 × 109/L), and his condition has been stable for several years while he has been receiving combination antiretroviral therapy. • He smokes two packs of cigarettes daily and stopped using IV drugs 10 years ago.
  • 46. • He denies fever, chills, and night sweats, but lost 18 lb (8 kg) since the onset of the illness. Results of a chest radiograph and representative CT scan images are shown.
  • 47. Which is the most likely diagnosis? A. Pneumocystis jiroveci pneumonia. B. Kaposi sarcoma. C. Aspergillosis. D. Adenocarcinoma
  • 48. • The radiograph shows an abnormal opacity in the lingula and enlargement of the left hilum, and the CT scan images show mass lesions in the lingula, hilum, and mediastinum, with encasement of the left upper lobe bronchus. These findings are most consistent with advanced lung cancer (choice D is correct).
  • 49. • Although Kaposi sarcoma, non-Hodgkin lymphoma, and cervical cancer are recognized as AIDS-defining diseases in people who are HIV-seropositive. • The incidence of lung cancer is now recognized to be increased in people with HIV infection, especially in the years following the use of combination antiretroviral therapy as the standard of care.
  • 50. • Although most people with HIV infection are current or former smokers, their risk of developing lung cancer appears to be increased, even when incidence ratios are adjusted for smoking. • The prevalence of microsatellite alternations reflecting genomic instability also occurs with greatly increased frequency in HIV-associated lung cancers, possibly playing a role in their pathogenesis
  • 51. • Patients with lung cancer in the setting of HIV infection tend to be relatively young at presentation (mean age, 45 years) and have mild or moderate immunosuppression. • Similar to age-matched control subjects, 75% to 90% present with stage III or IV disease. Adenocarcinoma is the most common histologic type, and the prognosis appears to be worse in patients with HIV infection. • Until further studies are performed, the diagnostic and therapeutic approaches to HIV-associated lung cancer are the same as for other patients, although the efficacy and toxicity of chemotherapy and radiotherapy may be different.
  • 52. • Until further studies are performed, the diagnostic and therapeutic approaches to HIV- associated lung cancer are the same as for other patients, although the efficacy and toxicity of chemotherapy and radiotherapy may be different.
  • 53. • Pneumocystis pneumonia is the most common opportunistic infection in patients with HIV infection but would not be expected to present with lung and mediastinal masses or in a patient with a CD4 lymphocyte count 200/mL ( 0.200 × 109/L) (choice A is incorrect).
  • 54. • Kaposi sarcoma may appear with multiple pulmonary nodules, pleural effusions, and intrathoracic lymphadenopathy, but this pattern of a single large lung mass with unilateral lymphadenopathy would be distinctly unusual with this neoplasm (choice B is incorrect).
  • 55. • Invasive pulmonary aspergillosis occurs in patients with HIV infection but usually appears with invasive and cavitary disease, without hilar and mediastinal lymphadenopathy, and only in the setting of severe immunosuppression (CD4 lymphocyte count usually 50/mL [ 0.050 × 109/L]) (choice C is incorrect).
  • 56. Which is the most likely diagnosis? A. Pneumocystis jiroveci pneumonia. B. Kaposi sarcoma. C. Aspergillosis. D. Adenocarcinoma
  • 57. Case No. 3
  • 58. • A 29-year-old man with a 2 pack-year smoking history is referred for evaluation of an abnormal chest radiograph.
  • 59. • The patient had a prolonged hospital admission 9 months ago for traumatic injuries related to a car accident. • A representative chest radiograph early in that hospitalization
  • 60. • He suffered traumatic brain injury that left him unable to communicate and wheelchair- bound but able to move all four extremities. The patient’s past history is notable for community-acquired pneumonia 4 weeks prior to the car accident, for which he was treated as an outpatient.
  • 61. • Several CT scans have been performed over the last 5 months, with the most recent noting that the lesion in question has increased in size from 5.6 × 4 cm to 7.3 × 5 cm over the 2- month interval.
  • 62. • The patient’s mother notes that he currently has no discernible respiratory signs other than an occasional nonproductive cough. Results of an examination of his lungs are normal. Which of the following is the most appropriate next step? A. Transthoracic needle biopsy. B. Bronchoscopy with biopsy. C. Surgical biopsy. D. Observation
  • 63. • The patient has a traumatic rupture of the right hemidiaphragm with herniation of the liver into the right chest cavity. • Continued observation is the most appropriate current choice, given the patient’s lack of symptoms and a clear diagnosis of liver herniation made by chest CT scan (choice D is correct).
  • 64. • Given a clear diagnosis, no invasive diagnostic procedures are required (choices A, B, and C are incorrect). A. Transthoracic needle biopsy. B. Bronchoscopy with biopsy. C. Surgical biopsy.
  • 65. • In this patient, the increasing size of the lesion indicates gradual entrance of the liver though a right hemidiaphragm defect driven by the pleuroperitoneal pressure gradient. The CT scan indicates that the enlarging chest lesion is liver, as supported by very similar Hounsfield units (density) of the “chest” lesion and the liver, and the contiguous nature of the chest lesion and the liver
  • 66. • Right-sided hemidiaphragm ruptures/tears are less frequently accompanied by herniated viscera than are left-sided ruptures. • The viscera most commonly herniated are the liver, as in this patient, and, occasionally, the colon. • Right sided hemidiaphragm trauma-related tears (defects) are eight times less common than left-sided tears, likely due to the protective effect afforded to the right hemidiaphragm by the liver.
  • 67. • Blunt trauma due to motor vehicle accidents is the most common cause of traumatic closed rupture of either hemidiaphragm, as was the case with this patient. • When the diagnosis of right-sided rupture is not made immediately, diagnosis can be delayed from weeks, as in this patient, to years.
  • 68. • With left-sided traumatic diaphragmatic rupture, herniation of the stomach, spleen, large bowel, liver, small intestines, and omentum is common. • Intrathoracic splenosis and spread of splenic tissue to the chest cavity can occur after traumatic injuries to the spleen. • Nonsurgical diagnosis of traumatic diaphragmatic herniation can often be made by plain chest radiograph. • CT scan or MRI imaging may be needed to confirm herniation
  • 69. Case No. 4
  • 70. • A 54-year-old woman was referred for assessment of neck swelling. She went to her family physician a few weeks ago and was diagnosed with hypertension. She started receiving indapamide. • Shortly thereafter, she noted swelling on the right side of her neck and a rash over the anterior chest. She saw her family physician who noted swelling on the right side of her neck. • He arranged a chest radiograph followed by a CT scan of the neck and chest. She had a 30 pack-year smoking history. She had no other medical problems. Results of a physical examination were negative other than the neck swelling.
  • 71. At this point, what would you do? • A. Start low-molecular-weight heparin. • B. Arrange early radiotherapy. • C. Discontinue indapamide. • D. Arrange urgent chemotherapy.
  • 72. • The chest radiograph demonstrates a right lung mass. The CT scan shows a 3.0 × 2.5-cm mass in the posterior segment of the right upper lobe and a 2.9 × 2.0-cm right hilar lymph node. • The right internal jugular vein reveals thrombosis, with edema surrounding it. The thrombus extends inferiorly to the right brachiocephalic vein, but the superior vena cava is patent. Ultimately, the patient was shown to have non-small cell lung cancer. This patient, therefore, has a malignancy with internal jugular vein thrombosis (IJVT).
  • 73. What is the next step? • A. Start low-molecular-weight heparin. • B. Arrange early radiotherapy. • C. Discontinue indapamide. • D. Arrange urgent chemotherapy.
  • 74. • Early radiotherapy would be appropriate if the patient had a superior vena cava obstruction, but that is not the case (choice B is incorrect). • Discontinuing indapamide would be appropriate if the neck swelling and rash were thought to be due to an allergic reaction. However, the swelling and “rash,” related to collateral blood supply, were secondary to the jugular vein thrombosis (choice C is incorrect). • An oncology consult should be scheduled, but the administration of chemotherapy should be delayed until a tissue diagnosis is obtained. Chemotherapy is not urgently required in this clinical setting (choice D is incorrect).
  • 75. Case No. 5
  • 76. • A healthy, active 82-year-old woman who regularly hikes at 14,000-ft (4,200-m) elevation develops a urinary tract infection, for which she is treated with nitrofurantoin. Within 3 days, she develops acute respiratory insufficiency requiring emergent intubation and mechanical ventilation support. • After a thorough evaluation, she was diagnosed with acute nitrofurantoin pulmonary toxicity and empirically treated with IV corticosteroids with symptomatic improvement and extubation after 10 days. • After completing a 4-week taper of oral corticosteroids, she develops progressive breathlessness, without exertional hypoxia, while walking 500 to 1,000 ft (150 to 300 m). • Follow-up CT imaging of the chest demonstrated resolution of the previously identified ground-glass opacities without residual parenchymal abnormalities. Spirometry was recently performed. • In addition to breathlessness, she notes significant anxiety when walking, with a globus sensation and inability to get a satisfying breath.
  • 77. The next most appropriate intervention for the patient would be? • A. PET scan. • B. CT angiography. • C. Cardiopulmonary exercise testing. • D. Fiberoptic laryngoscopy.
  • 78. variable, inspiratory obstruction fixed inspiratory and expiratory obstruction
  • 79. • Laryngotracheal stenosis, when associated with a prior injury such as intubation, manifests with symptoms 2 to 4 weeks following the initial injury. Dyspnea, air hunger, hoarseness, stridor, dysphagia, or recurrent aspiration may occur. Signs of a fixed lesion on flow-volume loop may be present in patients with severe disease. Confirmation by direct laryngoscopy and bronchoscopy is required (choice D is correct). • Anterior glottic scarring is often extensive, involving the true vocal cords and false cords, and often is the result of unmanaged severe external laryngeal trauma. Posterior glottic stenosis is typically a manifestation of endotracheal tube injury. CT imaging is complimentary in preoperatively evaluating patients but is limited in grading the severity of the stenosis. Functional abnormalities of the vocal folds, termed vocal cord dysfunction, may occur after intubation and manifest with a globus sensation and paroxysmal dyspnea.
  • 80. • In the absence of a concern for primary or metastatic laryngeal obstruction, PET scan imaging has limited value in the evaluation of patients with laryngeal stenosis. Given this patient’s presentation, malignancy is not a primary concern (choice A is incorrect). • CT angiography and cardiopulmonary exercise testing are important modalities in the evaluation of patients with dyspnea. Given the abnormalities on the patient’s flow-volume curve, thromboembolic disease and progressive parenchymal involvement from the nitrofurantoin pulmonary toxicity are less likely (choices B and C are incorrect).
  • 81. The next most appropriate intervention for the patient would be? • A. PET scan. • B. CT angiography. • C. Cardiopulmonary exercise testing. • D. Fiberoptic laryngoscopy.
  • 82. Case No. 6
  • 83. • A 27-year-old woman, gravida 2, para 1, at 35 weeks of gestation, has dyspnea on exertion. She has had some element of dyspnea on exertion since late in her first trimester of pregnancy but feels that it has worsened since a car trip of approximately 2½ h in each direction. She now feels “winded” climbing the single flight of stairs in her house. She also has some discomfort in her right anterior lower chest with deep inspiration, which is new since the trip. She is still able to complete all of her daily activities and denies cough, wheeze, fever, or hemoptysis. There is no leg swelling or pain. Her past medical history is unremarkable, and she had no difficulties with her first pregnancy. There is no personal or family history of VTE. Her only medication is prenatal vitamin pills and she does not smoke. She reports no allergies.
  • 84. • On physical examination, she is in mild respiratory distress at rest. BP is 96/52 mm Hg, heart rate is 102/min, respiratory rate is 20/min, and temperature is 37.1C, orally. Oxygen saturation is 95% at rest, and falls to 91% with ambulation of approximately 200 ft (60 m). No jugular venous distension is present, and there is no chest wall tenderness at the right-side site of her chest where she has pain with inspiration. Breath sounds are normal, and no adventitious sounds are appreciated. Cardiac examination reveals tachycardia with regular rate and rhythm, and without rubs, murmurs, or gallops appreciated. Her abdomen is normal for gestational age, and there is no lower extremity edema. A chest radiograph performed with shielding shows some atelectasis at the right base, and lower extremity ultrasonography shows no evidence of DVT. The physician remains concerned about the possibility of pulmonary embolism and wishes to pursue the diagnosis further.
  • 85. Which diagnostic test is most appropriate at this point? • A. CT scan of the chest with contrast. • B. Measurement of plasma D-dimer concentration. • C. Transesophageal echocardiography. • D. V / Q scanning.
  • 86. • This patient has worsened subjective dyspnea, pleuritic chest pain, and exertional desaturation in a clinical setting, which could indicate pulmonary embolism (ie, occurring during the third trimester of pregnancy and following a period of relative immobilization for her automobile trip). • Dyspnea is very commonly experienced during pregnancy. This physiologic “dyspnea of pregnancy” appears due, in large part, to elevated concentrations of progesterone directly stimulating CNS respiratory centers to produce hyperventilation. This tends to develop early in the course of pregnancy and is not associated with chest pain or desaturation. In contrast, the risk for VTE progressively increases during pregnancy, peaking in the third trimester and the postpartum period. Because of the timing, setting, and associated features of her presentation, evaluation for VTE is warranted. • A chest CT scan with contrast is the diagnostic study of choice for patients with a high pretest probability of pulmonary embolism, such as this patient, or for patients who have an elevated plasma D- dimer concentration on initial testing.
  • 87. • A normal D-dimer concentration measured by a validated enzyme-linked immunosorbent assay (ELISA) method effectively excludes the diagnosis in a hemodynamically stable outpatient with low or intermediate clinical probability. • However, the D-dimer assay is of limited value in patients with a high pretest probability of pulmonary embolism, and has reduced specificity in pregnant patients, hospitalized patients, individuals with cancer, and the elderly. In this • patient, the already high pretest probability and the low specificity of the D-dimer assay during pregnancy render the D-dimer test unhelpful.
  • 88. • V/Q scanning is a reasonable alternative in institutions that do not have adequate experience in the use of CT imaging in pulmonary embolism. However, V/Q scanning is diagnostic in only 30% to 50% of patients with suspected pulmonary embolism, and the remainder need to be followed up with additional studies. In addition, while the maternal radiation dose of V/Q scanning is less than CT scanning, the fetal radiation dose can actually be higher. The greatest utility of V/Q imaging generally is among patients with a low pretest probability of pulmonary embolism and a normal chest radiograph. In such a setting, a normal or near normal perfusion scan is generally sufficient to exclude the diagnosis; however, this patient had right lower lobe atelectasis, making a definitive study unlikely. For all of these reasons, V/Q imaging is undesirable in this patient. If the chest radiograph findings were normal in the patient, a perfusion scan would have been useful in excluding pulmonary embolism if it returned as normal or near normal.
  • 89. • In hemodynamically unstable patients in whom pulmonary embolism is suspected, echocardiography can presumptively establish the diagnosis by demonstrating right-sided heart strain (visible via transthoracic or transesophageal techniques) and/or by demonstrating emboli in the main pulmonary arteries (generally only visible via transesophageal studies). Echocardiography offers the advantage of being a bedside procedure that can be performed and interpreted quickly in patients who are too ill to permit transport, and it does not require either ionizing radiation or contrast material. However, it is unlikely to be diagnostic in this hemodynamically stable patient. • MRI is an attractive option because it does not use ionizing radiation to generate images. To date, no health risks have been associated with MRI, although experience with its use in pregnancy is not extensive. Unfortunately, the utility of MRI to diagnose pulmonary embolism is limited by insufficient sensitivity and a high rate of technically inadequate images.
  • 90. Which diagnostic test is most appropriate at this point? • A. CT scan of the chest with contrast. • B. Measurement of plasma D-dimer concentration. • C. Transesophageal echocardiography. • D. V / Q scanning.