Relapse rate and renal prognosis in ANCA-associated vasculitis according to long-term ANCA patterns

Abstract

Long-term observation of patients with ANCA-associated vasculitis (AAV) allows the identification of different longitudinal patterns of ANCA levels during follow-up. This study aimed to characterize these patterns and to determine their prognostic significance. All ANCA determinations performed in two university hospitals during a 2-year period were retrospectively reviewed. Patients were included in the analysis if they had high titers of anti-myeloperoxidase (anti-MPO) or anti-proteinase 3 (anti-PR3) antibodies at least once, ≥ 5 serial ANCA determinations and AAV diagnosed by biopsy or American College of Rheumatology (ACR) classification criteria. Patients' time-course ANCA patterns were classified as monophasic, remitting, recurrent or persistent. Associations between ANCA patterns and prognostic variables (relapse rate and renal outcome) were analysed by univariate and multivariate statistics. A total of 99 patients [55 with microscopic polyangiitis (MPA), 36 with granulomatosis with polyangiitis (GPA) and eight with eosinophilic granulomatosis with polyangiitis (EGPA)] were included. Median follow-up was 9 years. Among patients diagnosed with MPA or GPA, recurrent or persistent ANCA patterns were associated with a higher risk of clinical relapse [hazard ratio (HR) = 3·7, 95% confidence interval (CI) = 1·5-9·1 and HR = 2·9, 95% CI = 1·1-8·0, respectively], independently of clinical diagnosis or ANCA specificity. In patients with anti-MPO antibodies, the recurrent ANCA pattern was associated with worsening renal function [odds ratio (OR) = 5·7, 95% CI = 1·2-26·0]. Recurrent or persistent ANCA patterns are associated with a higher risk of clinical relapse. A recurrent ANCA pattern was associated with worsening renal function in anti-MPO-associated vasculitis.

Keywords: anti-neutrophil cytoplasmic antibodies; prognosis; renal failure; vasculitis.

Fig. 1

Clinical relapse according to anti‐neutrophil cytoplasmic antibody (ANCA) pattern in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). (a) All ANCA vasculitis; (b) anti‐PR3 vasculitis; (c) anti‐myeloperoxidase (MPO) vasculitis.

Fig. 2

Worsening renal failure according to anti‐neutrophil cytoplasmic antibody (ANCA) pattern in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) anti‐myeloperoxidase (MPO) vasculitis.