Sequential therapy with belimumab followed by rituximab in Sjögren's syndrome associated with B-cell lymphoproliferation and overexpression of BAFF: evidence for long-term efficacy.

摘要: Objectives The overexpression of B-cell activating factor (BAFF) in mucosa-associated lymphoid tissue (MALT) may decrease the efficacy rituximab treatment Sjogren's syndrome (SS). Anti-CD20 therapy was effective on marginal zone B cells, murine model for human CD20 expression only when preceded by anti-BAFF therapy. possible a sequential anti-BAFF/anti-CD20 SS investigated. Methods We treated with belimumab, monoclonal antibody, and soon after patient severe, refractory SS, parotid low-grade MALT lymphoma cryoglobulinaemic vasculitis. Previous treatments plus high dose glucocorticoids, as well cyclophosphamide, azathioprine, plasma exchange, hyperbaric therapy, VAC prostacyclin, mycophenolate mofetil surgery, had previously failed. Treatment belimumab then given, but it also A new course (375 mg/m2; four weekly infusions) started 49 days last infusion belimumab. Results This belimumab-rituximab followed marked amelioration, complete persistent regression healing skin ulcer. full cycle repeated 6 12 months later; no further given following 22 up to now. Serum cryoglobulins rheumatoid became persistently negative serum BAFF C4 normal. No relevant side effects were noticed, except IgM. follow is now three half years. Conclusions Therapy be SS-related lymphoproliferation. safety or concomitant targeting deserves evaluation SS.