Clinical significance of ANCA positivity in patients with IgA vasculitis: a retrospective monocentric study.

We assessed the detection rate of antineutrophil cytoplasmic antibody (ANCA) and investigated the clinical significance of ANCA positivity at diagnosis in patients with IgA vasculitis (Henoch-Schönlein purpura). We retrospectively reviewed their medical records of 86 IgA vasculitis patients. We divided IgA vasculitis patients based on ANCA positivity and compared variables at diagnosis and poor outcomes and medication during follow-up between the two groups. All-cause mortality, relapse, chronic kidney disease (CKD) (stage 3-5) and end-stage renal disease (ESRD) were defined as poor outcomes. We assessed the renal histological features based on the International Study of Kidney Disease in Children (ISKDC) classification and Oxford classification. Comparison of cumulative survivals was analysed by the Kaplan-Meier survival analysis. Five of 86 IgA vasculitis patients (5.8%) had ANCA and all ANCA-positive patients had myeloperoxidase (MPO)-ANCA. IgA vasculitis patients with ANCA exhibited pulmonary and nervous involvement of IgA vasculitis more frequently than those without. There was no significant difference in renal involvement between the two groups. There were no significant differences in renal histological features and poor outcomes related to renal function between IgA vasculitis patients with and without ANCA. In addition, 5 IgA vasculitis patients did not meet the classification criteria for ANCA-associated vasculitis (AAV). Particularly, there were no significant differences in CKD and ESRD-free survival rates between IgA vasculitis patients with and without ANCA. 5.8% of IgA vasculitis patients had MPO-ANCA and poor outcomes of IgA vasculitis were not affected by the presence of ANCA.