A Review of Interstitial Lung Diseases. Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco
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1 A Review of Interstitial Lung Diseases Paul J. Wolters, MD Associate Professor Department of Medicine University of California San Francisco
2 Outline Overview of diagnosis in ILD Why it is important Definition/Classification High resolution CT scan Multidisciplinary approach Cases Interactive format (ARS) Review of radiology and pathology Questions/Discussion
3 Why it is Important to be Aware of ILD s
4 Don t stop with pulmonary fibrosis Reasons for a specific diagnosis: Many forms are treatable Treatments depend on diagnosis Prognosis varies Eligibility for clinical trials
5 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
6 Clinical History Idiopathic usual interstitial pneumonitis (UIP) = IPF nonspecific interstitial pneumonitis (NSIP) desquamative interstitial pneumonitis (DIP) respiratory bronchiolitis ILD (RBILD) acute interstitial pneumonitis (AIP) Unclassified Drugs Collagen Vascular Joint c/o Sicca sx Skin rash Raynaud s Occupational/Environmental -Asbestos -Birds/Molds/Organic Material (HP)
7 Exam: Clues to diagnosis Lung: Crackles on exam. Findings c/w sarcoid: Uveitis, skin rash, erythema nodosum, hepatomegaly. Findings c/w CVD: Synovitis, rash, sclerodactaly. Findings c/w IPF: Clubbing
8 CXR is Not Useful for Differentiating ILD s HP IPF CVD-ILD NSIP
9 High resolution CT (HRCT) mm collimation Images taken every 10 mm Supine, prone and expiratory images
10 HRCT is the Key to Diagnosing ILD s Pattern of abnormality on HRCT scan may suggest a specific ILD. HRCT findings guide subsequent diagnostic tests. HRCT findings may be sufficient for diagnosis.
11 Usual Interstitial Pneumonia (UIP) Irregular lines in a patchy, basilar, subpleural distribution Traction bronchiectasis minimal Ground glass opacities. Honeycomb lung
12 Nonspecific Interstitial Pneumonitis (NSIP) Ground glass opacities (100%) Consolidation (98%) Nodules (96%) Traction bronchiectasis (95%) Intralobular reticulation (87%) Lower lobe predominance
13 Desquamative Interstitial Pneumonia (DIP) Ground glass opacity with a basilar, subpleural, and lower lobe distribution Lower lobe reticular opacities
14 Hypersensitivity Pneumonitis Subacute phase : multifocal or diffuse GGO poorly defined centrilobular nodules Subacute and chronic phases : mosaic perfusion air trapping on expiratory images CT may be normal in some cases
15 Sarcoidosis Perilymphatic nodules Peribronchial nodules GGO Upper lobe predominant, +/- fibrosis
16 Diagnostic Algorithm Suspected PF Detailed history, PE + HRCT + PFTs/Labs Diagnostic Non-diagnostic 1. IPF 2. Sarcoidosis 3. CV-ILD 4. Hypersensitivity pneumonitis 5. Rare disease (LAM, PAP) Lung biopsy
17 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:
18 Case 1
19 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
20 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
21 Case 1 PFTs FVC 5.10 (83%) FEV (90%) FEV1/FVC 0.87 TLC 7.40 (88%) DLCO (57%)
22 CXR
23 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
24 HRCT
25 HRCT
26 HRCT
27 HRCT
28 HRCT
29 HRCT
30 HRCT
31 Serological evaluation ANA negative RF = 4
32 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
33 VATS
34 VATS
35 Usual Interstitial Pneumonia (UIP) Temporal heterogeneity Subpleural accentuation of fibrosis Regions of normal lung Microscopic honeycombing Fibroblast Foci
36 Diagnosis of IPF Management Enrolled in clinical trial Pulmonary rehabilitation Weight loss (if overweight) Lung transplant referral
37 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:
38 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:
39 Case 2
40 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
41 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
42 CXR
43 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
44 HRCT
45 HRCT
46 HRCT
47 HRCT
48 HRCT
49 HRCT
50 Serological evaluation ANA 1:80, speckled RF = 18, cyclic citrullinated antibody (CCP) negative
51 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
52 VATS
53 VATS
54 VATS
55 Nonspecific Interstitial Pneumonitis (NSIP) Uniform involvement of the lung with Septal thickening Interstitial inflammation (lymphocytes) +/ Fibrosis
56 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
57 Additional serologies RNP negative Scl70 negative SSA, SSB negative Jo 1 negative, CPK 104
58 Diagnosis of NSIP Management Treated with prednisone and mycophenolate (CellCept) Pulmonary rehabilitation
59 NSIP Distinguishing NSIP from UIP is a challenge! Patients usually younger, more likely to be women Honeycombing on HRCT is uncommon in NSIP Surgical biopsy shows diffuse thickening of alveolar septae and few if any fibroblast foci
60 IPF NSIP
61 IPF NSIP
62 IPF NSIP
63 NSIP The diagnosis of NSIP should prompt you to go back to look for an etiology Occult connective tissue disease Drug reaction Hypersensitivity pneumonitis
64 Case 3
65 Case 3 43 year old man Subacute progressive dyspnea PMH: mild obstructive sleep apnea on CPAP 6 cm H20, seasonal allergies Meds: cetirizine, multivitamin
66 Case 3 Social: non smoker, banker, no known exposures, no pets. Family: no history of ILD BP 116/56, hr 60, rr16, 98% RA (88% walking) Moderately obese Lungs clear to auscultation
67 Case 3 PFTs: FVC 3.17 (73%) FEV (77%) FEV1/FVC 0.77 TLC 4.65 (82%) DLCO 15.1 (58%)
68 CXR
69 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
70 HRCT
71 HRCT
72 HRCT
73 HRCT
74 HRCT
75 HRCT
76 HRCT expiratory views
77 HRCT expiratory views
78 HRCT expiratory views
79 Serological Evaluation ANA negative RF 12 SS A, SS B negative
80 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
81 VATS Biopsy: Low Power
82 VATS Biopsy: High Power
83 VATS Biopsy: High Power
84 Hypersensitivity Pneumonitis Airwaycentric inflammation Poorly formed granuloma (giant cell) Areas of organizing pneumonia
85 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
86 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. Additional history
87 Hypersensitivity Pneumonitis Management Remove antigen (Relocated from new home) Treat with prednisone Pulmonary rehabilitation
88 Thank you!
89 UCSF Interstitial Lung Disease Clinic Clinical Coordinator: (415) Clinic fax: (415)
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