Rituximab in relapsing and de novo MPO ANCA-associated vasculitis with severe renal involvement: a case series

Abstract

Background Antineutrophil cytoplasmic antibody associated vasculitis (AAV) is a group of diseases associated in most cases with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs). Rituximab- based remission induction has been proven effective in ANCA associated vasculitis but scarce data exist in forms with severe renal involvement. In this case series, we report the outcomes in patients with de novo or recurrent MPO-AAV and severe renal involvement treated with rituximab without cyclophosphamide (CYC). Methods In this single centre retrospective study, we analysed patients with a clinical diagnosis of de novo or recurrent AAV who met the following criteria: detection of P-ANCA, creatinine clearance lower than 30 ml/min, induction of remission therapy with rituximab without concomitant CYC and a follow up period of at least 6 months. The primary outcomes were complete remission after induction therapy, renal function recovery and mortality after the induction treatment. Results Eight patients met the inclusion criteria. The M:F ratio was 1:7, the average age was 54 years old and the median follow up was 10 months (7–72); in 2 patients there was a MPA renal limited vasculitis. A renal biopsy was performed in 7 patients. The median BVAS score at rituximab induction was 14(range 6–21). Two patients required haemodialysis before the induction treatment. Four patients developed end stage renal disease (ESRD) that required haemodialysis. These data show a remission of the disease, associated with a stabilization of the kidney function in 50% of patients. In 3 patients who did not show a response, there was also no response to CYC. Conclusions This study shows a partial efficacy of rituximab in renal function recovery and a low risk of infectious complications in patients with MPO vasculitis with severe renal involvement, in particular in the short term. The optimal treatment in this subgroup of patients still has to be established because data are lacking.

The Efficacy and Safety of Rituximab in ANCA-Associated Vasculitis: A Systematic Review

“…In this study, RTX provided an incomplete renal function improvement. Lack of efficacy was shown in cases that were previous non-responders and RTX recovery function was not related to the reaction to CYC [ 140 ]. Roccatello et al [ 132 ] studied 25 AAV patients and compared the efficacy of two regimens, including RTX+CYC+GC without maintenance therapy (RTX group) and CYC+GC with AZA maintenance therapy (conventional group).…”

“…In a study, RTX was not found effective in the improvement of patients’ otolaryngological complaints [ 120 ]. Caroti et al [ 140 ] studied AAV patients with severe renal disease and showed that three of eight patients did not respond to the RTX. Lovric et al [ 35 ], in their study, showed that one in 15 patients with upper-airway and eye involvement did not respond to RTX.…”

Evidence-based treatment of ANCA-associated vasculitis with kidney involvement

“…В исследовании RITUXVAS, напротив, участвовали пациенты с тяжелым АНЦА-ГН, а ритуксимаб не уступал ЦФ в отношении восстановления СКФ. Сопоставимая эффек тивность ритуксимаба и ЦФ у пациентов с тяжелым АНЦА-ГН (СКФ менее 20 мл/мин, в том числе диализзависимая почечная недостаточность) была также продемонстрирована в отдельных сериях наблюдений и ретроспективных исследованиях [31]. В исследовании RITUXVAS введению ритуксимаба предшествовали две инфузии ЦФ, однако в настоящее время в обычной клинической практике подобное сочетание этих препаратов не используется.…”