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.

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

“…Although this has been previously reported in the literature (4,5,7,8,(27)(28)(29)(30)(31), we analyzed CT images in sequential hospital-based patients before they received treatment and determined the prevalence of each imaging component of ILD (Table 2). Ground-glass opacity was the most prevalent component, which indicates that a large proportion of MPA patients have an active inflammatory process in the lungs before they receive treatment.…”

Pulmonary fibrosis presenting as an early manifestation of microscopic polyangiitis

“…Of the 59 wellidentified cases in the literature with pulmonary fibrosis and MPA, PF had been manifested prior to the development of MPA in 23, MPA preceded the presence of PF in 1 and both conditions were identified concurrently in 35 [6,[11][12][13][14][15][16]. Nevertheless, it is possible that, in patients in whom both diseases were diagnosed at the same time, PF might have been present earlier but not clinically apparent.…”

A case of pulmonary fibrosis associated with rheumatoid arthritis, scleroderma sine scleroderma and ANCA associated vasculitis.

“…Nozu et al demonstrated that a high titer of ANCA is associated with a poor prognosis and higher risk for progression to MPA (Nozu et al 2009). In fact, in a study by Foulan et al, 41% of patients with PF and ANCA positivity developed MPA over time (Foulon et al 2008).…”

“…Patients with initial diagnosis of PF, occasionally acquire seropositivity for MPO-ANCA, which may develop to microscopic polyangitis (MPA) (Ando et al 2013). The prevalence of ANCA in patients with ILD in literature is suggested at 8-36% (Nozu et al 2009;Foulon et al 2008). Homma et al showed positive ANCA as an unfavorable prognostic factor in patients with pulmonary fibrosis (Homma et al 2004).…”