Case Presentations in ILD. Harold R. Collard, MD Department of Medicine University of California San Francisco

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1 Case Presentations in ILD Harold R. Collard, MD Department of Medicine University of California San Francisco

2 Outline Overview of diagnosis in ILD Definition/Classification High-resolution CT scan Multidisciplinary approach Cases Interactive format (ARS) Review of radiology and pathology Questions/Discussion

3 Don t stop with pulmonary fibrosis Reasons for a specific diagnosis: Many forms are treatable Treatments depend on diagnosis Prognosis varies Clinical trial eligibility requirements

4 Clinical Classification Pulmonary Fibrosis Exposure-related: - Occupational - Environmental - Avocational - Medication Desquamative interstitial pneumonia (DIP) Acute interstitial pneumonia (AIP) Nonspecific interstitial pneumonia (NSIP) Idiopathic interstitial pneumonia (IIP) Idiopathic pulmonary fibrosis (IPF) Connective tissue disease: - Scleroderma - Rheum. arthritis - Sjogrens - UCTD Respiratory bronchiolitis interstitial lung dis. (RBILD) Cryptogenic organizing pneumonia (COP) Lymphocytic interstitial pneumonia (LIP) Other: -Sarcoidosis - Vasculitis/Diffuse alveolar hemorrhage (DAH) - Langherhans cell histiocytosis (LCH) - Lymphagioleiomyomatosis (LAM) - Pulmonary alveolar proteinosis (PAP) - Eosinophilic pneumonias - Neurofibromatosis - Inherited disorders - Chronic aspiration - Inflammatory bowel disease

5 Diagnostic Algorithm Suspected PF Detailed history + PFTs/Labs + HRCT Smoking history Occ/Env history Autoimmune ROS Family history Spirometry Lung volumes/dlco ANA, RF

6 High-resolution CT (HRCT) mm collimation Images taken every 10 mm Supine, prone and expiratory images

7 Reticular abnormality

8 Honeycomb cysts

9 Ground glass

10 Centrilobular nodules

11 Diagnostic Algorithm Suspected PF Detailed history + PFTs/Labs + HRCT Diagnostic 1. IPF 2. Sarcoidosis 3. CV-ILD 4. Hypersensitivity pneumonitis 5. Rare disease (LAM, PAP) Non-diagnostic Lung biopsy

12 Multidisciplinary approach Agreement increased with multidisciplinary approach Step Assessment Method Information Provided Agreement (κ) Individual HRCT 2 Individual 3 4 Discussion (clinician and radiologist) Individual (clinician, radiologist and pathologist) HRCT, clinical data HRCT, clinical data HRCT, clinical data, SLB Discussion HRCT, clinical data, SLB Step Modified from: Flaherty KR, et al. Am J Respir Crit Care Med. 2004;170:

13 Case 1

14 Case 1 57 year old man Abnormal CXR 3 months prior to presentation (notes normal CXR from 5 years ago) Mild dyspnea on exertion, no cough PMH: GERD for 30 years Meds: omeprazole 40mg daily

15 Case 1 Social: lifetime non-smoker, some mold in home, pharmacist Family: no history of ILD 114/73 hr 70 rr 16 96% RA (93% walking) Dry inspiratory crackles at both bases Normal cardiac examination No clubbing, joint deformities, rashes

16 Case 1 PFTs FVC 5.10 (83%) FEV (90%) FEV1/FVC 0.87 TLC 7.40 (88%) DLCO (57%)

17 CXR

18 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serological evaluation 4. High resolution CT scan 5. Surgical lung biopsy 6. No additional testing; diagnosis is clear

19 HRCT

20 HRCT

21 HRCT

22 HRCT

23 HRCT

24 HRCT

25 HRCT

26

27 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serological evaluation 4. Surgical lung biopsy 5. No additional testing; diagnosis is clear

28 Serological evaluation ANA negative R F = 4

29

30 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Surgical lung biopsy 4. No additional testing; diagnosis is clear

31 VATS

32 VATS

33 VATS

34

35 What is this pathologic pattern? Usual interstitial pneumonia Non-specific interstitial pneumonia Diffuse alveolar damage Organizing pneumonia Peribronchiolar inflammation with granuloma formation

36 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. No additional testing; diagnosis is clear

37 No additional testing performed

38 What is your diagnosis 1. Pulmonary fibrosis 2. Cryptogenic organizing pneumonia 3. Idiopathic pulmonary fibrosis 4. Non-specific interstitial pneumonia 5. Asbestosis 6. Other

39 Management Diagnosis of IPF Enrolled in clinical trial Pulmonary rehabilitation Lung transplant referral

40 IPF: Published Definition A specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of usual interstitial pneumonia on surgical lung biopsy. ATS/ERS. Am J Respir Crit Care Med. 2000;161:

41 Survival Median survival for IPF is 2 3 years No proven therapy IPF Modified from Bjoraker JA. Am J Respir Crit Care Med. 1998;157:

42 Case 2

43 Case 2 63-year-old woman 5-month history of shortness of breath and nonproductive cough PMH: hypertension, treated for TB exposure in 1980, arthritis Medications: diltiazem

44 Case 2 Social: former smoker (18 pk/yrs); office worker. Family: No history of ILD 135/80 hr 90 rr 14 94% RA (90% walking) Dry inspiratory crackles at both bases Normal cardiac examination No clubbing, joint deformities, rashes

45 Case 2 PFTs FVC 3.10 (63%) FEV (70%) FEV1/FVC 0.86 TLC 5.40 (72%) DLCO (48%)

46 CXR

47 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serological evaluation 4. High resolution CT scan 5. Surgical lung biopsy 6. No additional testing; diagnosis is clear

48 HRCT

49 HRCT

50 HRCT

51 HRCT

52 HRCT

53 HRCT

54

55 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serological evaluation 4. Surgical lung biopsy 5. No additional testing; diagnosis is clear

56 Serological evaluation ANA 1:80, speckled RF = 18, cyclic citrullinated antibody (CCP) negative

57

58 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Surgical lung biopsy 4. No additional testing; diagnosis is clear

59 VATS

60 VATS

61 VATS

62 VATS

63

64 What is this pathologic pattern? Usual interstitial pneumonia Non-specific interstitial pneumonia Diffuse alveolar damage Organizing pneumonia Peribronchiolar inflammation with granuloma formation

65 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Additional serologies 4. No additional testing; diagnosis is clear

66 Additional serologies RNP negative Scl70 negative SSA, SSB negative Jo-1 negative, CPK 104

67 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. No additional testing; diagnosis is clear

68 No additional testing performed

69 What is your diagnosis 1. Connective-tissue related pulmonary fibrosis 2. Cryptogenic organizing pneumonia 3. Idiopathic pulmonary fibrosis 4. Non-specific interstitial pneumonia 5. Hypersensitivity pneumonitis 6. Other

70 Management Diagnosis of NSIP Treated with prednisone and mycophenolate (CellCept) Pulmonary rehabilitation

71 NSIP Distinguishing NSIP from UIP is a challenge! Patients usually younger, more likely to be women Honeycombing on HRCT uncommon in NSIP Surgical biopsy shows diffuse thickening of alveolar septae and few if any fibroblast foci

72 IPF/NSIP

73 IPF/NSIP

74 IPF/NSIP

75 NSIP The diagnosis of NSIP should prompt you to go back to look for an etiology Occult connective tissue disease Drug reaction Hypersensitivity pneumonitis

76 Case 3

77 Case 3 43 year old man Subacute progressive dyspnea PMH: mild obstructive sleep apnea on CPAP 6 cm H20, seasonal allergies Meds: cetirizine, multivitamin

78 Case 3 Social: non-smoker, banker, no known exposures Family: no history of ILD BP 116/56, hr 60, rr16, 98% RA (88% walking) Moderately obese Lungs clear to auscultation

79 Case 3 PFTs: FVC 3.17 (73%) FEV (77%) FEV1/FVC 0.77 TLC 4.65 (82%) DLCO 15.1 (58%)

80 CXR

81 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Echocardiogram 4. High resolution CT scan 5. Surgical lung biopsy 6. No additional testing; diagnosis is clear

82 HRCT

83 HRCT

84 HRCT

85 HRCT

86 HRCT

87 HRCT

88 HRCT expiratory views

89 HRCT expiratory views

90 HRCT expiratory views

91

92 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serological evaluation 4. Serum precipitins 5. Surgical lung biopsy 6. No additional testing; diagnosis is clear

93 Serological Evaluation ANA negative R F 12 SS-A, SS-B negative

94

95 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. BAL and transbronchial biopsy 3. Serum precipitins 4. Surgical lung biopsy 5. No additional testing; diagnosis is clear

96 VATS Biopsy: Low Power

97 VATS Biopsy: High Power

98 VATS Biopsy: High Power

99 VATS Biopsy: High Power

100 What is this pathologic pattern? Usual interstitial pneumonia Non-specific interstitial pneumonia Diffuse alveolar damage Organizing pneumonia Peribronchiolar inflammation with granuloma formation

101 What additional testing would you perform next in this patient? 1. Bronchoalveolar lavage (BAL) 2. Sputum culture 3. Serum precipitins 4. Additional history 5. No additional testing; diagnosis is clear

102 Additional history Patient recently moved to a new home built on the site of a former pigeon farm. There is ongoing construction of new units around his home and lots of dust.

103 What is your diagnosis 1. Non-specific interstitial pneumonia 2. Cryptogenic organizing pneumonia 3. Idiopathic pulmonary fibrosis 4. Hypersensitivity pneumonitis 5. Sarcoid 6. Other

104 Management Diagnosis of Hypersensitivity pneumonitis Treated with prednisone Relocated from new home Pulmonary rehabilitation

105 Hypersensitivity Pneumonitis

106 Thank you!

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