Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality
Year: 2022
Type: article
Study design: Observational Study
Abstract: OBJECTIVE: To evaluate the prevalence, clinical and radiographic features, and long-term outcomes of interstitial lung disease (ILD) in a United States-based ANCA-associated vasculitis (AAV) cohort. METHODS: In this retrospective cohort study, we identified cases of ILD within the 2002-2019 Mass General Brigham AAV Cohort, a consecutive inception cohort of PR3- or MPO-ANCA+ AAV patients. ILD diagnosis and classification as fibrotic or non-fibrotic were confirmed by review of available chest imaging by two board-certified radiologists. Cox proportional hazard models, with age as the time scale, were used to estimate the association of AAV-ILD with all-cause mortality. RESULTS: Of 684 patients in the MGB AAV Cohort, 91 (13%) had ILD which preceded the diagnosis of AAV by a mean of 2.2 years. AAV-ILD patients were older (67 vs 60 years, P < 0.001) than patients without ILD but the distribution of sex and race was similar. AAV-ILD patients were more often MPO-ANCA+ (93% vs 65%, P < 0.001); among MPO-ANCA+ patients (n = 470), 85 (18%) had ILD. The majority of ILD was fibrotic (76%) and UIP was the most common ILD pattern (42%). The baseline forced vital capacity (FVC) % predicted among ILD patients was 81 ± 20%. Fibrotic AAV-ILD was associated with a 58% higher risk of death (aHR 1.58, 95% CI 1.06, 2.37) compared with AAV patients without ILD. CONCLUSION: ILD is a frequent complication of AAV, especially MPO-ANCA+ AAV, often preceding recognition of AAV. Fibrotic AAV-ILD is associated with a higher risk of death than AAV without ILD.
Source: Lara D. Veeken
Authors: Brett Doliner, Karen Rodriguez, Sydney B Montesi, Xiaoqing Fu, Amita Sharma, Zachary S Wallace
Institutions: Harvard University, Massachusetts General Hospital
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: green
Awards: Personalizing AAV Management by Leveraging Big Data: Targeting Complication Clusters, Vascular Leak and Outcomes in IPF, G5715663391, In vivo endobronchial OCT for IPF diagnosis and therapy response assessment, Impact of ANCA Type and Rituximab vs. Cyclophosphamide on Cardiovascular Risk, Mortality, and Quality-Adjusted Life Years inANCA-Associated Vasculitis
OpenAlex ID: W4282836477