Document Detail

Interstitial pneumonia associated with MPO-ANCA: Clinicopathological features of nine patients.
MedLine Citation:
PMID:  22992339     Owner:  NLM     Status:  Publisher    
Myeloperoxidase anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) is a well known marker for small vessel vasculitis. Recent reports have demonstrated that interstitial pneumonia (IP) may rarely be associated with serum MPO-ANCA. Yet, little is known about the histological features. We reviewed surgical lung biopsy from nine patients with IP of uncertain etiology with serum MPO-ANCA. There was a male predominance (6:3) with a median age of 62.1. Histologically, eight patients presented with a usual interstitial pneumonia (UIP) pattern of pulmonary fibrosis, frequently accompanied by areas of nonspecific interstitial pneumonia (NSIP) pattern. One patient showed diffuse alveolar damage (DAD), and two patients showed mixture of UIP and DAD reflecting acute exacerbation of UIP. Microscopic honeycomb cysts were common, but fibroblastic foci were inconspicuous. The most frequent additional findings were small airway disease (9/9), and lymphoid follicles (7/9). Neither capillaritis nor vasculitis was seen in any of our cases. Three patients had microscopic hematuria, but none progressed to microscopic polyangiitis during the follow up. Mortality rate was 44% (median follow up 39.1 months). IP associated with MPO-ANCA showed characteristic histology dominated by UIP pattern. Vasculitis was not identified in our cohort, but small airways disease and lymphoid follicles were present in most cases. IP associated with MPO-ANCA may be a histologically distinctive disease from idiopathic pulmonary fibrosis. Mortality was relatively high and life threatening acute exacerbation may occur.
Tomonori Tanaka; Kyoko Otani; Ryoko Egashira; Yukio Kashima; Hiroyuki Taniguchi; Yasuhiro Kondoh; Kensuke Kataoka; Akira Shiraki; Yasuhiko Kitasato; Kevin O Leslie; Junya Fukuoka
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-9-17
Journal Detail:
Title:  Respiratory medicine     Volume:  -     ISSN:  1532-3064     ISO Abbreviation:  Respir Med     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-9-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8908438     Medline TA:  Respir Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Ltd. All rights reserved.
Laboratory of Pathology, Toyama University Hospital, Toyama, Japan.
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