Active Immunological Profile Is Associated with Systemic Sjögren’s Syndrome

Background The aim of this paper was to study the evolution of primary Sjögren’s syndrome (pSS) immunological profile, its impact on pSS activity and long-term evolution in a bicentric cohort of French patients with pSS ( n = 445, mean age 53.6 ± 14 years, mean follow-up 76.1 ± 51 months). Methods This is a retrospective cohort study. Results Two hundred twelve patients were Sjögren’s syndrome A (SSA) positive, and 131 were both SSA and Sjögren’s syndrome B (SSB) positive. Sixty-eight patients (15%) had cryoglobulinemia. Active systemic profile (i.e., hypergammaglobulinemia, rheumatoid factor (RF), and anti-Sjögren’s syndrome A (anti-SSA), anti-Sjögren’s syndrome B (anti-SSB) positivity), associated with multisystemic involvement, leads to an increased utilization of corticosteroid and hydroxychloroquine. Multivariate analysis pointed out independent statistical association between hypergammaglobulinemia, anti-SSA, anti-SSB, and RF. Cryoglobulinemia is associated with multi-systemic involvement, lymphoma, and pSS-related death. Conclusion The subset of patients with active immunological profile is characterized by systemic complications leading to immunosuppressive drug utilization and polyclonal B-cell activation profile.