Abstract and Introduction
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.
Introduction
Peripheral nervous system (PNS) involvement is one of the extraglandular features observed in primary Sjögren's syndrome (pSS). Various forms of neuropathy, including pure sensory polyneuropathy, mixed sensorimotor polyneuropathy and multineuritis, have been described. Vasculitis has been shown to be associated with better response to treatment and outcome.
Recently, rituximab (RTX) was used in pSS, and some studies suggested its efficacy. No study has aimed to evaluate the effect of RTX on pSS with PNS involvement. We evaluated the safety and efficacy of RTX in pSS with PNS involvement, using the data of the French Autoimmunity and Rituximab (AIR) registry.