Pulmonary Board Review Workshop-1

1. Pulmonary Board Review Workshop-1 Joaquim S.Tavares,MD,FCCP,FAASM

2. Questions to review • 2,11,12,20,46,49,58,65,82,87,89,103

3. A 73 y/o female has a 2 week hx of worsening dyspnea and dry cough.She has not had fever or any recent travel.Idiopathic pulmonary fibrosis was diagnosed 2 years ago by open lung biopsy. She also has hx of HTN and GERD.Her medications are prednisone,diltiazem,HCTZ,and omeprazole. • On PE,she is afebrile,the blood pressure is 142/86, Oxygen saturation with the patient breathing oxygen,10 L/mn by face mask is 90%. There are dry crackles at the lung bases.Cardiac and abdominal examinations are normal

4. Gram Stain of the sputum is negative.;culture is pending.CT scan of the chest is negative for PE but shows new areas of alveolar infiltrates superimposed on previous areas of honeycombing changes. • Which of the following is the most appropriate next text in te evaluation: • A-Bronchoscopy with lavage • B-Fungal serologies • C-Right heart catheterization • D-Swallowing evaluation

5. A 71 y/o woman has a 3-week hx of mild pain in the shoulders and thighs and weakness when rising from a seated position.She also has a new rash on her hands.Eight months ago she was evaluated for dyspnea and new interstitial lung infiltrates that resulted in a lung biopsy and a diagnosis of idiopathic interstitial pneumonia.She was treated with prednisone,60mg/day,for 1 month;the dose was then tapered to 10mg/day. • PE: swelling and discoloration of the eyelids and an erythematous scaly rash over the extensor surfaces

6. Of interphalangeal joints of both hands.There is symmetric weakness of the proximal hip flexors and shoulder girdle muscles.Hand strength is normal • ANA positive at 1:1280,CPK=1270 and ESR=60mm/h • Which of the following is the most appropriate management for this patient? • A-Electromyography • B—Repeat lung biopsy • C-Skin biopsy • D-Taper prednisone dosage.

7. If you see hands,rashes,HRCT • Anti-synthetase antibodies • Anti J0-1 is most common • TX: PO:0.5-0.75mg/kg/d for 2-6 weeks,taper over 4-12 weeks • IV: 1 g solumedrol once daily x 3 months. • Cytotoxic agent: CYC,AZA

8. A 74 y/o man is evaluated for a 5 year hx of gradually progressive dyspnea and dry cough without wheezing or hemoptysis. For the past 2 years he has had pain and occasional swelling in both knees.He has not had fever or lost weight.He smoked one pack of cigarettes a day from age 18 to 60 years.He worked as an insulator for 40 years. PE shows no clubbing or cyanosis. Auscultation of the lungs reveals bilateral end-inspiratory crackles.PFT’s: TLC=67% of predicted. RV=72% predicted;FEV1/FVC=89% predicted; DLCO=52% predicted

10. Which of the following is the most likely diagnosis? • A-Asbestosis • B-Idiopathic Pulmonary Fibrosis • C-Rheumatoid Interstitial lung disease • D-Pulmonary Sarcoidosis

11. A 36 y/o woman with an 18-month history of diffuse cutaneous systemic sclerosis is evaluated for a 6-month hx of dry cough and decrease in exercise capacity.She is otherwise healthy and takes no medications. • PE:RR=18/mn; PFT’s: FEV1 of 80%,FVC of 76% and DLCO of 83%. Hrct shows bilateral reticular infiltrates with a basilar and peripheral distribuition.There is no ground-glass opacification. Visual impression of the extent of lung involvement is 10%

12. Which of the following is the most appropriate management for this patient’s pulmonary disease? • A-Cyclophosphamide and low dose prednisone • B-High dose prednisone • C-Open lung biopsy • D-Periodic HRCT and PFT’s

13. A 64 y/o woman is evaluated for a 6-week hx of dyspnea,dry cough,fever,chills,night sweats,and fatigue,which have not responded to treatment with azithromycin and levofloxacin;she has lost 2.2 kg(5lbs) during that time. • A thorough examination 6 months ago while she was asymptomatic,including laboratory/chest radiograph was normal.She has never smoked,has no known environmental exposures,and has not traveled recently Her only medications are aspirin and multivitamins

14. PE: Temperature is 37.8;other vital signs are normal;There are scattered crackles in the mid-lung zones.There is no clubbing and skin examination is normal. • Which of the following is the most likely diagnosis? • A)Cryptogenic Organizing pneumonia • B)Idiopathic pulmonary fibrosis • C)Lymphocytic interstitial pneumonia • D)Nonspecific interstitial pneumonia

18. A 36 year-old woman is evaluated in the office after having had a small(less than 2cm) spontaneous pneumothorax. The patient had a 6-month hx of worsening dyspnea and dry cough. She has never smoked and has a hx of bilateral breast augmentation.PE: no clubbing or crackles. • Which of the following is the most likely Dx? • A)Emphysema B)IPF C)Lymphangioleiomyomatosis D)-Pulmonary Langherans cell histiocytosis

20. LAM • Cystic lung disease exclusively in women of child-bearing age. • Proliferation of abnormal smooth muscle-like cells • Common in Tuberous sclerosis complex • Hemoptysis,chylothorax,cough,dyspnea • BX: stain for HMB-45 • Tx:Medroxyprogesterone,Rapamycin.

21. Tuberous Sclerosis • Autosomal dominant • 2 genetic loci: TSC1,TSC2 • Similar lung findings with LAM • Sing tags, seizures • Tx: 02,bronchodilators, lung transplant.

22. Lymphomatoid Granulomatosis • Eosinophilic Granuloma,Histiocytosis X,Langerhans cell Histiocytosis. • Males,smokers; mixed obstructive/restrictive lung • Dx: Birbeck granules on electron microscopy; positive stain for S-100,CD1a Ag. • BX: Langerhan’s cell histiocytes • Tx: 02,steroids; vinblastin.

24. A 77 y/o woman is evaluated for a 1 year hx of progressive dyspnea and dry cough.She has had previous evaluations for these symptoms and has had abnormal chest radiographs and HRCT scans. She also has Hx oh heart failure,systolic HTN,Sjogren syndrome,and monoclonal gammopathy of unknown significance;her medications are metoprolol,lisinopril,furosemide,hypromellose artificial tears,potassium and a multivitamin. • 3 weeks ago,she had a open lung biopsy that showed polyclonal plasma-cell infiltration of the pulmonary

25. Interstitium within the alveolar septa with fibrotic changes,including rare honeycombing consistent with lymphocytic interstitial pneumonia.HRCT showed scattered ground-glass opacification,rare subpleural reticular changes and tiny pulm nodules. PE:ankle edema.no digital clubbing. Which of the following is the most likely diagnosis? • A)Connective tissue disease related LIP • B)Idiopathic LIP • C)IPF • D)Primary pulmonary lymphoma

26. A 60 y/o woman is evaluated for progressive shortness of breath and a dry cough.She was diagnosed with idiopathic pulmonary fibrosis 3 years ago. • PE: resting hypoxemia,clubbing of the fingers;late inspiratory crackles.Chest radiographs shows decreased lung volumes and bilateral basal opacities,and patchy honeycombing. • Pulmonary function testing shows:significantly decreased FVC,TLC,and FRC;DLC0 is 35% of predicted.There has been a decrement in the FVC greater than 10% in the last 6 months.

27. Which of the following is the best treatment option for this patient? • A)Interferon gamma • B)Lung transplantation • C)N-acetylcysteine • D)Prednisone and azathioprine

29. A 55 y/o woman with a long standing Hx of moderate to severe asthma is evaluated for worsening productive cough,dyspnea,and wheezing.She has not had fever,chills,or chest pain.Her current medications are low-dose inhaled corticosteroids,a long acting beta agonist,and albuterol. • PE:bilateral wheezing, and scattered ronchi over the upper lung fields. • Chest radiograph shows patchy infiltrates in both upper lobes and bronchiectasis. HRCT:central bronchiectasis and mucous plugging.

30. Which of the following is the most appropriate next step in the evaluation of this patient? • A)Bronchoscopy with lung biopsy • B)Methacoline challenge test • C)Positron-emission tomography • D)Skin test for Aspergillus fumigatus • E)Sweat chloride test

31. A 28 y/o man is evaluated for an abnormal chest radiograph done for chronic intermittent nonproductive cough of 6 months duration.The radiograph showed bilateral hilar lymphadenopathy and normal lung parenchyma.The patient has fatigue and intermittent mild central chest discomfort when he coughs.He has not had weight loss,fever,night sweats,or recent respiratory illness.There is no cervical or axillary lymphadenopathy. • Labs: serum calcium is 10.8mg/dl. CT: bilateral hilar and mediastinal lymphadenopathy,with pulm nodules

32. Which of the following is the most appropriate next test in the evaluation of this patient? • A)Bone marrow biopsy • B)Bronchoscopy with mediastinal lymph node and lung biopsy • C)Mediastinoscopy • D)Serum Histoplas antibody testing

34. A 44 y/o man is evaluated for a 4-month Hx of progressive dyspnea and cough.The patient is otherwise healthy and takes no medications.He has smoked 2 packs of cigarettes a day for 20 years. • PE:vital signs are normal;pulmonary function tests show a mixed obstructive-restrictive defect.HRCT of the chest shows bibasilar reticular or reticulonodular abnormalities in a centrilobular distribution with diffuse areas of ground-glass opacification.

35. Most likely diagnosis?

36. Which of the following is the most likely diagnosis? • A)Cryptogenic organizing pneumonia • B)Idiopathic pulmonary fibrosis • C)Nonspecific interstitial pneumonia’ • D)Respiratory bronchiolitis interstitial lung disease

38. PAP • Rare disorder • Autoantibodies against GM-CSF • BAL:acellular globules PAS positive • Tx: WLL, GM-CSF

40. Case 1 • A 25 y/o woman presents with increasing dyspnea on exertion. The patient has a history of epilepsy.On physical exam,several hypomelanotic patches are present on the thorax,and several periungal fibromas are noted on the fingers and toes. CT of the chest as follows. Which is the most likely diagnosis • 1-Lymphomatoid Granulomatosis(EG) • 2-Tuberous sclerosis • 3-Sarcoidosis • 4-IPF • 5-LAM

41. Interstitial Lung Diseases • Occupational/Environm • Iatrogenic/Drug induced • Granulomatous(Sarcoid/HP) • Collagen Vascular • Idiopathic Interstitial Pneumonias • Inherited • Unique Entities

42. Unique Entities • Alveolar Proteinosis • Langherans Cell Granulomatosis • Lymphangiomyomatosis

43. Inherited • Hermansky-Pudlak Syndrome • Tuberous Sclerosis • Neurofibromatosis • Metabolic Storage Disorder • Familial ILD

44. Idiopathic Interstitial Pneumonias(IIP) • A-Idiopathic Pulmonary Fibrosis(IPF)-UIP • B-OTHERS: • 1- RBILD(DIP) • 2-BOOP(COP) • 3-NSIP • 4-AIP • 5-LIP

45. Environmental/Occupational/Medications • Miners: Pneumoconiosis • Sandblasters/Granite: Silicosis • Welders/shipyards/pipefitters:Asbestosis • Farm Workers: Hypersensitivity Pneumonitis • Poultry/Bird fanciers: Hypersensitivity Pneumonitis • Aerospace,nuclear,computers:Berylliosis • Amiodarone,chemotx,etc

46. History of Present Illness • Acute symptoms in the absence of infection: COP(BOOP), AIP, AEP, Drug Induced, HP. • Chronic syptoms: IPF,NSIP,chronic HP,CTD. • Hemoptysis: Goodpasture,,Vasculitis, Wegener’s • Wheezing:ABPA,Churg Strauss,CEP.

47. Extrapulmonary Symptoms • GERD: Scleroderma related ILD,IPF • Occular symptoms:Sarcoidosis,CTD, HLA-B27 related • Sinusitis: Wegener’s • Diabetes Insipidus: Sarcoidosis, Pulmonary LCG(EG) • Epilepsy or Mental Retardation: Tuberous Sclerosis.

48. Demographics and Family History • IPF: >60 y/a • EG(Pulm LCG):young,smokers • RB-ILD and DIP: almost exclusively in smokers. • LAM: women on childbearing age. • Strong family history:IPF

49. Diagnostic Tests • ANA,RF,ESR • C,P-ANCA • Peripheral blood Lymphocyte proliferation test(Beryllium)

50. Chest Radiographs • Upper lobe predominance: Sarcoidosis,Berylliosis,EG,Silliosis,Ankylosing Spond. • Lower Lobes:IPFchronic HP,SS,Asbestosis • Pneumothorax: LAM,pulmonary LCG(EG)