Scleroderma

Dimitrios Daoussis, Stamatis-Nick C. Liossis, Georgios Yiannopoulos, and Andrew P. Andonopoulos
Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Rion, 26504 Patras, Greece
Received 24 February 2011; Revised 4 June 2011; Accepted 7 June 2011

Systemic sclerosis (SSc) is a systemic rheumatic disease characterized by vasculopathy, autoimmunity, and fibrosis. The available therapeutic options are extremely limited and prognosis is variable. Cyclophosphamide (CYC) has shown modest efficacy in the treatment of SSc-associated interstitial lung disease (ILD) but its long-term use is accompanied by significant toxicity. Therefore, novel therapeutic approaches are desperately needed. During the last decade, B-cell depletion by rituximab (RTX), a monoclonal antibody that targets B-cells, has emerged as a promising therapy for a wide range of systemic autoimmune diseases.

It has been approved for the treatment of rheumatoid arthritis but it has also been tried in systemic lupus erythematosus, systemic vasculitides, and multiple sclerosis, among others. An expanding body of experimental evidence suggests that Bcells play a role in the fibrotic process, raising the question of whether B-cell depletion might be a potential therapeutic approach in SSc. During the last 2 years, 4 small-scale, open-label studies and a few case reports have addressed this question to some extent, reporting encouraging results. In this paper we provide the experimental evidence supporting the active role of B-cells in fibrosis and summarize all the available clinical evidence regarding the use of RTX in patients with SSc.