Rituximab for Primary Angiitis of the Central Nervous System: Report of 2 Patients from the French COVAC Cohort and Review of the Literature

Primary Angiitis of the Central Nervous System in Adults and Children

“…7 Tumor necrosis factor (TNF) blockers have been reported anecdotally to be useful in 2 cases of PACNS, 45 and rituximab in 3 cases. 46,47 We suspect that the off-label use of biologics, particularly anti-TNF drugs and rituximab, extends well beyond the literature (eg, we have used anti-TNF drugs in 2 patients with recurrent PACNS), but no conclusions about their absolute or relative effectiveness can be drawn.…”

“…Given the rarity of this condition, these trials require multicenter collaboration efforts. In advance of such studies, ongoing publication of case series 12 and even case reports [45][46][47][48] is still of considerable value.…”

Adult Primary Central Nervous System Vasculitis Treatment and Course: Analysis of One Hundred Sixty‐Three Patients

“…Two other patients were treated with tumor necrosis factor a blockers (for disease refractory to treatment and for relapsing vasculitis, respectively). Therefore, although the experience is limited to few case reports (19)(20)(21), biologic agents may represent a useful therapeutic option for patients who are intolerant to conventional immunosuppressive agents or as second-line therapy for patients with treatment-refractory disease or relapses.…”

Rituximab treatment in primary angiitis of the central nervous system

“…Treatment options for refractory or relapsed disease are limited. Individual cases and small case series have reported the successful use of biological therapy with rituximab (anti‐CD20 targeted monoclonal antibody) and infliximab (anti‐TNFα targeted monoclonal antibody) for this condition . Much of the experience of treating vasculitis with rituximab comes from use in AAV, where in randomised controlled trials rituximab is as effective as cyclophosphamide for induction therapy and possibly superior for treatment of relapsed disease .…”