Abstract
Systemic sclerosis (SSc) is a systemic rheumatic disease with poor prognosis since therapeutic options are limited. Recent evidence from animal models suggests that B-cells may be actively involved in the fibrotic process. B-cells from tight skin mice, an animal model of scleroderma, display a “hyperresponsive” phenotype; treatment with rituximab (RTX) significantly attenuates skin fibrosis in this animal model. In humans, B-cell infiltration is a prominent finding in most lung biopsies obtained from patients with SSc-associated interstitial lung disease. Several open label studies have assessed the clinical efficacy of RTX in SSc. In most patients skin fibrosis improved; lung function either improved or remained stable. Definite conclusions regarding the clinical efficacy of RTX in SSc cannot be drawn but further exploration with a multicenter, randomized study is warranted.
Highlights
Systemic sclerosis (SSc) is a systemic rheumatic disease characterized by vasculopathy, autoimmunity, and fibrosis
tight skin mouse (TSK) mice are characterized by extensive skin fibrosis and immunological abnormalities including the presence of autoantibodies to topoisomerase-1, both reminiscent of those observed in the human disease [9]
It has been reported that TSK B-cells exhibit enhanced CD19 signaling compared to WT B-cells, the expression of this molecule was similar in TSK and WT B-cells [10]
Summary
Systemic sclerosis (SSc) is a systemic rheumatic disease characterized by vasculopathy, autoimmunity, and fibrosis. 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 been tried in systemic lupus erythematosus [2], systemic vasculitides [3], and multiple sclerosis [4], 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 [5,6,7,8].
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