Present and novel biologic drugs in primary Sjögren's syndrome

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by xerostomia and xerophthalmia. In at least one-third of patients, the disease may be complicated by extraglandular involvement. Due to the lack of evidence-based recommendations, current therapeutic options for pSS are mainly empirical, often reflecting their use in other autoimmune diseases. Nevertheless, recent advances in the understanding of pathogenic pathways in pSS encourage the belief that blocking them may be of value in the treatment of the disease. Despite failing to demonstrate efficacy in clinical trials, because of the well-established role of B-lymphocytes in the pathogenesis of pSS, rituximab has been the most frequently used to date, but with much less success than in the treatment of patients with rheumatoid arthritis, vasculitis and lupus. However, in the last few years a number of other biologics have been developed and are under investigation. The aim of this article is to review the use of biologic therapies in pSS.