B-Cell Depletion Therapy in Systemic Sclerosis : Experimental Rationale and Update on Clinical Evidence

Abstract EN

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.