title: ANCA-associated lung fibrosis: analysis of 17 patients.
abstract: 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.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Bichat-Claude Bernard, Service de Pneumologie A, Paris, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Foulon
authors.forename: Philippe
authors.initials: P
authors.lastname: Delaval
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
authors.forename: Benoît
authors.initials: B
authors.lastname: Wallaert
authors.forename: Marie-Pierre
authors.initials: MP
authors.lastname: Debray
authors.forename: Michel
authors.initials: M
authors.lastname: Brauner
authors.forename: Pascale
authors.initials: P
authors.lastname: Nicaise
authors.forename: Jacques
authors.initials: J
authors.lastname: Cadranel
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.forename: Michel
authors.initials: M
authors.lastname: Aubier
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001992:Bronchoalveolar Lavage Fluid / Q000276:immunology; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D011658:Pulmonary Fibrosis / Q000150:complications / Q000276:immunology* / Q000401:mortality; D012189:Retrospective Studies; D012907:Smoking; D018709:Statistics, Nonparametric; D015996:Survival Rate; D014657:Vasculitis / Q000150:complications / Q000276:immunology* / Q000401:mortality
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase