Clinical utility of proteinase 3-antineutrophil cytoplasmic antibody at diagnosis in predicting subsequent relapse in patients with microscopic polyangiitis

Abstract

Background In real clinical practice, patients classified as microscopic polyangiitis (MPA) according to the new 2022 criteria, despite proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) positivity, are occasionally encountered. Hence, this study investigated the clinical utility of PR3-ANCA detected at diagnosis in MPA patients.Methods We included 191 patients with MPA in this study and retrospectively reviewed their medical records, in which clinical data at diagnosis and during follow-up were recorded. Additionally, we investigated three major poor complications of MPA, such as subsequent all-cause mortality (ACM), relapse, and end-stage kidney disease (ESKD). Cumulative survival rates were compared among the groups using Kaplan-Meier survival analysis with the log-rank test.Results At diagnosis, the median age of 191 MPA patients was 64 years (68 males and 123 females). PR3-ANCA was detected in only 10 patients (5.2%). During follow-up, among the 191 MPA patients, 32 patients died, 43 patients experienced subsequent relapse, and 39 progressed to ESKD. In the comparative analyses, at diagnosis, PR3-ANCA-positive MPA patients were younger and had lower white blood cell count and blood urea nitrogen levels than PR3-ANCA-negative patients. During follow-up, PR3-ANCA-positive MPA patients showed a higher frequency of subsequent relapse than PR3-ANCA-negative patients. Additionally, in Kaplan-Meier survival analysis, PR3-ANCA-positive MPA patients exhibited a significantly lower relapse-free survival rate than PR3-ANCA-negative patients over the follow-up period.Conclusion In this study, we found that PR3-ANCA positivity at diagnosis is significantly associated with subsequent relapse in patients with MPA during follow-up.