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1 Idiopathic versus secondary Usual Interstitial Pneumonia (UIP) pattern in a series of 96 consecutive surgical lung biopsies: The value of histologic ancillary findings in a multidisciplinary discussion Maria Cecilia Mengoli, Gloria Montanari, Alberto Cavazza, Francesca Barbisan, Luisella Righi, Alessandro Marchioni, Paolo Spagnolo and Giulio Rossi COI: no conflicts of interest to declare Amsterdam, 6 September, 2017
2 Histologic Usual Interstitial Pneumonia (UIP) pattern GEOGRAPHIC and TEMPORAL HETEROGENEITY FIBROBLASTIC FOCI Courtesy of Tom Colby HONEYCOMBING
3 The availability of new effective drugs in IPF require an appropriate diagnosis
4 The Diagnosis of Idiopathic Pulmonary Fibrosis (IPF) Clinical Conditions associated with UIP pattern IDIOPATHIC PULMONARY FIBROSIS (IPF) CHRONIC HYPERSENSITIVITY PNEUMONITIS COLLAGEN VASCULAR DISEASE DRUG TOXICITY ASBESTOSIS FAMILIAL IPF HERMANSKY-PUDLAK SYNDROME Etc.
5 May the pathologist be helpful in discriminating between idiopathic and secondary UIP pattern?
6 UIP pattern ANCILLARY FINDINGS Courtesy of Tom Colby Granulomas Eosinophils Follicolar bronchiolitis Asbestos bodies Chronic pleuritis Chronic Hypersensitivity Pneumonitis Drug toxicity Rheumatoid Arthritis Asbestosis Connective Tissue Disease
7 Materials & Methods cases surgical lung biopsies and original dignosis of UIP exclusion 11 cases for «end-stage» fibrosis 2 cases for fibrosing NSIP 96 surgical lung biopsies with histologic UIP pattern ORIGINAL DIAGNOSIS Histologic examination for: Honeycombing +/- Heterogeneity Fibroblastic foci (1-3+) and..
8 Materials & Methods-2 «Ancillary Findings» SRIF Bridging Fibrosis Chronic Pleuritis Giant cells/granulomas Lymph-PC, Follicles +/-GC Metaplastic tissue PBM/Bronchiolcentric injury Eosinophils Proteinois-like material
9 Sex Age Materials & Methods-3 Clinical Domain Serological Domain Morphologic Domain Smoking habit Symptoms Respiratory function (spirometry, DLCO) Occupational/ Environmental exposure Comorbidity GERD PH Therapy Autoimmunity: FR, ANA, ENA Precipitins HRCT pattern Definite UIP pattern 8 cases (8%) Possible UIP pattern 34 cases (35%) *+ 17 Cases with only Thoracic RX Inconsistent with UIP pattern 37 cases (39%)
10 Materials & Methods-4 ORIGINAL DIAGNOSIS HISTOLOGIC DIAGNOSIS MULTIDISCIPLINARY CLIN-RAD-PATH DIAGNOSIS * PATHOLOGIC CONFIDENCE RATE (high/low) for the DIAGNOSIS of IDIOPATHIC vs SECONDARY UIP Clinical Domain Serological Domain Morphologic Domain Imaging Histology 1 STEP 2 STEP 3 STEP
11 Results-1 n % M/F (n=96) 71/25 (74/26) Smoking habit Lung Function ex-current/never/na 51/23/22 (53/24/23) restrictive/obstructive/mixed/normal/na 41/2/1/26/26 (43/2/1/27/27) Exposure Autoimmunity Comorbidity Country/City 8/88 (8/92) Work +/Hobby +/- 35/17/44 (36/18/46) +/-/NA 13/35/48 (13,5/36,5/50) Cancer 19 (20) GERD 11 (11,5) PH 2 (2) Not exp 46% Hobby exp 18% Work exp 36%
12 Results-2 18 case (19%) 31 cases (33%) 5 cases (5%) SRIF Bridging Fibrosis Chronic Pleuritis 32 cases (33%) 48 cases (50%) INF 7 cases (7%) GC 48 cases (50%) Giant cells/granulomas 41 cases (43%) Lymph-PC, Follicles +/-GC 5 cases (6%) Metaplastic tissue 15 cases (16%) PBM/Bronchiolcentric injury Eosinophils Proteinois-like material
13 ORIGINAL DIAGNOSIS HISTOLOGIC DIAGNOSIS CLIN-RAD-PATH DIAGNOSIS 82 cases (85%) Idiopathic UIP pattern/ipf 11 cases (12%) Chr HP 2 cases (2%) CTD-ILD 1 case (1%) Asbestosis 52 cases (54%) Idiopathic UIP pattern/ipf 14 cases (15%) Chr HP 3 cases (2%) CTD-ILD 26 cases (27%) Possible Secondary UIP 64 cases (67%) Idiopathic UIP pattern/ipf 22 cases (23%) Chr HP 6 cases (6%) CTD-ILD 3 cases (3%) Asbestosis 1 case (1%) Chr Sarcoid Chr HP 12% IDIOPATHIC / IPF 85% POSSIBLE SECONDARY UIP 27% Chr HP 15% IDIOPATHIC/ IPF 54% Chr HP 23% IDIOPATHIC /IPF 67%
14 CHRONIC HYPERSENSITIVITY PNEUMONITIS Giant cells/granulomas PBM/Bronchiolcentric injury Bridging Fibrosis p<0.001 p<0.001 p<0.001 CTD-ILD Lymph-PC, Follicles +/-GC Chronic Pleuritis SECONDARY UIP Proteinois-like material p=0.003 p<0.001 p=0.006
15 Results-5 The Multidisciplinary Approach Chr SARCOID ASBESTOSIS CTD-ILD 6 2 Datenreihen2 MDD Datenreihen1 Original diagnosis Chr HP IPF IPF Chr HP CTD-ILD
16 The Lancet Respir Med, patients originally diagnosed with IPF according to 2011 guidelines 43% chr HP 57% IPF 85% IPF (82 cases) MDD 19% Different Diagnosis (18 cases) 67% IPF (64 cases) 12% chr HP (11 cases) 4% CTD-ILD (4 cases) 2% Asbestosis (2 cases) 1% chr Sarcoid (1 cases)
17 Conclusion-1 - UIP pattern is the most common pattern among chronic interstitial pneumonia - UIP pattern is associated with IPF, but a subset of patients has a secondary disease The presence of some «ancillary findings»: 1. INTERSTITIAL GIANT CELLS/GRANULOMAS 2. PERIBRONCHIOLAR METAPLASIA 3. CHRONIC PLEURITIS 4. FOLLICLES WITH GERMINAL CENTERS 5. ALVEOLAR PROTEINOSIS-LIKE MATERIAL at histology is significantly associated with secondary UIP in Chronic HP and CTD-ILD but
18 Conclusion-2 THE MULTIDISCIPLINARY CLIN-RAD-PATH APPROACH still remains the DIAGNOSTIC GOLD STANDARD
19 MANY THANKS for YOUR ATTENTION!!!
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