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Efficacy of rituximab in primary Sjögren's syndrome with peripheral nervous system involvement: results from the AIR registry

Abstract

Objective To evaluate rituximab (RTX) in primary Sjögren's syndrome (pSS) with peripheral nervous system (PNS) involvement.

Methods Patients with pSS and PNS involvement who were included in the French AIR registry were analysed.

Results 17 patients (age 60 years (44–78 years); 14 were female) were analysed.

Neurological improvement was noted in 11 patients (65%) at 3 months. Rankin scale decreased from 3 (1–5) to 2 (1–5), 2 (1–5) and 2 (1–6) after 3, 6 and 9 months (p=0.02). European Sjögren's Syndrome Disease Activity Index decreased from 18 (10–44) to 11 (5–20), 11 (5–29) and 12 (5–30) after 3, 6 and 9 months (p<0.05).

RTX was effective in neurological involvement in 9/10 patients with vasculitis or cryoglobulinaemia (90%) (group 1) at 3 months and in 2/7 cases (29%) without cryoglobulinaemia and vasculitis (p=0.03). Rankin and European Sjögren's Syndrome Disease Activity Index scales decreased significantly in group 1.

Conclusion RTX seems effective in cryoglobulinaemia or vasculitis-related PNS involvement in pSS.

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