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

Abstract

Summary 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.

Anti-neutrophil cytoplasmic antibody–associated vasculitis complicated with diffuse alveolar haemorrhage and central nervous system vasculitis

“…A flare in vasculitis is often accompanied by an increase in the serum ANCA level. 7 However, the patient’s serum MPO-ANCA level was lower at admission than at the evaluation prior to this admission. We hypothesise that this decrease in the MPO-ANCA level was affected by pre-treatment with PSL in the former hospital.…”

Neutrophils in ANCA-associated vasculitis: Mechanisms and implications for management

“…The various clinical manifestations of AAV often include a wide range of symptoms and signs due to the disorders of general status and damage to the renal, respiratory, nervous, and other systems (Hunter et al, 2020). Moreover, some of the injury complications may be fatal to life, such as acute renal failure and alveolar hemorrhage (Quartuccio et al, 2020;Oristrell et al, 2021).…”

Anti-neutrophil cytoplasmic antibody patterns can predict clinical relapse in ANCA-associated vasculitis: overall population and subgroups

“…Until now, some risk factors, such as PR3-ANCA positivity, age>65, lower serum creatinine and cardiovascular system involvement, have been reported, but not confirmed by other studies. ANCA has been proposed as a predictor of relapse since its entry into clinical studies (7), but the prediction of relapse based on ANCA patterns remains controversial (8)(9)(10)(11)(12)(13). Thai et al confirmed clinical outcomes and ANCA levels coincided for only 60% of the granulomatosis with polyangiitis (GPA) patients (11).…”