Opinion statement
Primary Sjögren’s syndrome (pSS) is an autoimmune disease, showing a broad heterogeneity concerning the symptoms, the involved organs, as well as the prognosis. The clinical presentation, in the majority of patients, is limited to the mucosal surfaces, although some patients may show a systemic involvement and/or immunological bio-markers strongly associated with a higher risk to develop lymphoma. The assessment of disease activity, by using validated scores, at the time of pSS diagnosis, has been shown to be helpful in identifying patients requiring a closer follow-up and more-intensive therapeutic management. At present, evidence-based therapy for pSS is limited to symptomatic drugs for dryness, and the choice of immunosuppressive or biologic agent treatment is mainly based on their efficacy in other systemic autoimmune diseases, as well as expert opinion and uncontrolled studies. In recent years, given the central role of B-lymphocytes in pSS pathogenesis, B-cell depleting therapy with anti-CD20 monoclonal antibody rituximab (RTX) has shown promising, results in clinical trials. Although data present in literature do not allow to draw definitive conclusion on the efficacy of RTX, B-cell depletion may be considered a therapeutic option for a subgroup of pSS patients. In particular, RTX treatment seems to be effective in early active disease, mainly on glandular swelling and in systemic manifestations.