title: B-cell depletion therapy in systemic sclerosis: experimental rationale and update on clinical evidence.
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.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Rion, 26504 Patras, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Daoussis
authors.forename: Stamatis-Nick C
authors.initials: SN
authors.lastname: Liossis
authors.forename: Georgios
authors.initials: G
authors.lastname: Yiannopoulos
authors.forename: Andrew P
authors.initials: AP
authors.lastname: Andonopoulos