Document Detail

ANCA-associated lung fibrosis: analysis of 17 patients.
MedLine Citation:
PMID:  18640019     Owner:  NLM     Status:  MEDLINE    
In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients were past smokers, 3 current smokers and 6 non-smokers. Lung function tests at diagnosis were as follows (% predicted): total lung capacity 73%+/-18, vital capacity 82%+/-23, forced expiratory volume in 1 s (FEV(1)) 88%+/-24, carbon monoxide diffusion capacity of the lung 49%+/-2 (% predicted). Bronchoalveolar lavage results showed an increased cellularity with increased neutrophils counts. High resolution computed tomography of the chest showed prominent fibrosis with some degree of ground-glass attenuation in all patients. These characteristics were similar to the control group. Microscopic polyangiitis (MPA) was a major complicating event in ANCA-positive patients, occurring in 7 patients (anti-myeloperoxidase specificity in 5 patients). Pulmonary fibrosis predated occurrence of MPA in 6 patients and was diagnosed concomitantly with MPA in 1 patient. During the follow-up, 10/17 patients died. The death was directly related to vasculitis in 3 patients. We conclude that patients with pulmonary fibrosis should be evaluated for the presence of ANCA. Patients with positive ANCA testing, particularly if anti-myeloperoxidase, should be carefully monitored to detect the occurrence of microscopic polyangiitis.
Guillaume Foulon; Philippe Delaval; Dominique Valeyre; Benoît Wallaert; Marie-Pierre Debray; Michel Brauner; Pascale Nicaise; Jacques Cadranel; Vincent Cottin; Abdellatif Tazi; Michel Aubier; Bruno Crestani
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Publication Detail:
Type:  Journal Article     Date:  2008-07-18
Journal Detail:
Title:  Respiratory medicine     Volume:  102     ISSN:  1532-3064     ISO Abbreviation:  Respir Med     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-09-02     Completed Date:  2009-05-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8908438     Medline TA:  Respir Med     Country:  England    
Other Details:
Languages:  eng     Pagination:  1392-8     Citation Subset:  IM    
Assistance Publique-Hôpitaux de Paris, Hôpital Bichat-Claude Bernard, Service de Pneumologie A, Paris, France.
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MeSH Terms
Antibodies, Antineutrophil Cytoplasmic / blood*
Bronchoalveolar Lavage Fluid / immunology
Case-Control Studies
Kaplan-Meiers Estimate
Middle Aged
Neutrophils / immunology
Peroxidase / immunology
Pulmonary Fibrosis / complications,  immunology*,  mortality
Retrospective Studies
Statistics, Nonparametric
Survival Rate
Vasculitis / complications,  immunology*,  mortality
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic; EC

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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