ArticleRespiratory research2026
Prevalence and clinical outcomes of fibrotic interstitial lung disease in ANCA associated vasculitis: a single-centre, retrospective, cohort study.
Article in Respiratory research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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Who cites it
1 citing paper in PubMed.
- The Genetic Landscape of Fibrotic Interstitial Lung Diseases: Clinical Implications and Diagnostic Challenges in Familial Pulmonary Fibrosis.Journal of clinical medicine · 2026Review
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13 authors.
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Abstract
backgroundAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may affect multiple organs and interstitial lung disease (ILD) might be the first manifestation. Limited and discordant data are available on the prevalence of ILD in patients with AAV. Little is known about prognostic outcomes of these patients. The primary aim of the study was to determine the prevalence of fibrotic ILD in a cohort of AAV patients followed-up in a University Hospital in Italy. Clinical and prognostic outcomes of AAV-ILD were also assessed.
methodsData from 71 AAV patients (mean age 60.3 ± 16 years; female 58.9%) were retrospectively collected and analyzed.
resultsOverall ILD prevalence was 50.7%. Fibrotic ILDs represented 32.4% with a variable distribution according to AAV diagnosis: microscopic polyangiitis (MPA) 66.7%, eosinophilic granulomatosis with polyangiitis (EGPA) 41.1%, granulomatosis with polyangiitis (GPA) 31.8%. The presence of ILD was significantly different across AAV subgroups. Fibrotic ILD was more prevalent in MPA (60%) than EGPA (11.8%) and GPA (9.1%) and most frequently associated with usual interstitial pneumonia (UIP) radiologic pattern. The presence of ANCA was significantly associated with fibrotic ILDs (p = 0.01). In univariate analysis, only MPA increased the risk of having ILD (OR 2 (CI 95%: 0.96–4.67). Mortality was significantly higher in patients with ILD (p = 0.01), with fibrotic forms influencing this finding and MPA showing the highest mortality.
conclusionsFibrotic ILDs are common in patients with AAV and influence clinical and prognostic outcomes. MPA is the most frequent AAV associated to fibrotic ILD. ILD should be investigated at diagnosis in all patients with AAV, since early detection might impact the prognosis of these patients.
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