Off‐Trial Evaluation of the B cell–Targeting Treatment in the Refractory Cases of Antineutrophil Cytoplasmic Antibodies (ANCA)‐Associated Vasculitis: Long‐Term Follow‐Up from a Single Centre

Abstract

The aim of the study was to evaluate long‐term clinical and immunological effects of anti‐B cell treatment in patients with antineutrophil cytoplasmic antibodies (ANCA)‐associated vasculitis refractory to conventional immunosuppressive treatment. Rituximab (RTX) was added to the ongoing immunosuppressive treatment in 29 patients with refractory ANCA‐associated vasculitis. The disease activity was measured using Birmingham Vasculitis Activity Score/Wegener’s granulomatosis (BVAS/WG score), and clinical laboratory variables were recorded. The median BVAS/WG score before treatment was 6 (IQR 3–8), and 28 patients (97%) had disease flare classified either severe (62%) or limited (34%). Six of 29 patients (21%) achieved a complete remission, and 12 (41%) had a treatment response with ≥50% decrease in BVAS/WG score at 6 months. Fourteen patients (64%) with kidney involvement achieved remission, and in seven patients (50%), no flare was seen during the follow‐up period. Three patients had renal flare and were successfully re‐treated with RTX. Seventeen patients had disease symptoms from airways and eyes at RTX initiation, whereas only 29% displayed ≥50% treatment response. Limited clinical improvement was seen in patients with endobronchial lesions and trachea‐subglottic granulomatous disease. RTX is a potent therapeutic option for ANCA‐associated vasculitis refractory to conventional treatment. Best response may be expected in patients with vasculitic manifestations.

Long-term efficacy and safety of pre-emptive maintenance therapy with rituximab in Granulomatosis with Polyangiitis: Results from a single centre

“…The risk for severe infections seems mostly conditioned by important decreases in Ig especially after the first RTX round (as shown in our study) and concurrent medication such as CYC and MMF. Two studies report severe infection rates of 15.5 and 22.5 per 100 patient-years with a much shorter observation time with respectively 66% of the patients using CYC [19] and 75% using either CYC or MMF in combination with RTX [20]. Although there is no clear evidence for an increased risk of severe infections during long-term pre-emptive RTX maintenance, repeated treatment with RTX by further decreasing total immunoglobulins level as shown in our study and by influencing T cell immunity [21] can still potentially increase the risk for severe infections.…”

The 2016 European (Eular/Era-Edta) Recommendations for the Diagnosis and Management of Anca-Associated Systemic Vasculitis

“…РТМ эффективен у больных рефрактерным АНЦА-СВ, ранее получавших ЦФ, особенно в случаях ГН; вместе с тем периорбитальная гранулема хуже отвечает на лечение [23,24]. При недоступности РТМ предложено под наблю-дением экспертного центра заменять внутривенное введе-ние ЦФ на прием ЦФ внутрь [25].…”

Should rheumatoid factor in rheumatoid arthritis be sent to Davy Jones’s Locker?

“…Half of the patients have chronic renal disease [13,14]. Half of the patients receive RTX in monotherapy [13,14] while the others receive RTX in combination with other immunosuppressive drugs [29,31].…”

“…At least 6 AAV patients (1.2%) treated with RTX developed PCP among 516 patients from different cohorts [9][10][11][12][13][14][29][30][31][32] and proved fatal in 2 patients. PCP occurred 2, 3 and 32 months after the last RTX infusion in 3 patients including ours [13,29].…”