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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