Is there a role for B-cell depletion as therapy for scleroderma? A case report and review of the literature

Semin Arthritis Rheum. 2010 Oct;40(2):127-36. doi: 10.1016/j.semarthrit.2009.09.003. Epub 2009 Dec 11.


Objectives: Rituximab (RTX) has been successfully used in the treatment of several rheumatic diseases with an acceptable safety profile. We present herein a patient with systemic sclerosis (SSc) who exhibited significant improvement of his lung function and skin fibrosis following RTX administration, and review the literature regarding the role of B-cells in SSc and the potential efficacy of RTX in its treatment.

Methods: We performed an internet search using the keywords systemic sclerosis, scleroderma, rituximab, B-cells, fibrosis, interstitial lung disease (ILD), and therapy.

Results: Our patient, a 40-year old man with severe SSc-associated ILD, received 4 courses of RTX. The patient's lung function improved; forced vital capacity and diffusing capacity of carbon monoxide reached values of 35% and 33%, respectively, compared with 30% and 14% of pretreatment values. Skin thickening assessed clinically and histologically improved as well. Several lines of evidence suggest that B-cells may have a pathogenic role in SSc. B-cells from tight skin mice--an animal model of SSc--exhibit chronic hyperactivity; likewise, B-cells from patients with SSc overexpress CD19 and are chronically activated. Furthermore, studies have revealed that B-cell genes were specifically transcribed in SSc skin and that B-cell infiltration was a prominent feature of SSc-associated ILD. The potential clinical efficacy of RTX in SSc has been explored in a limited number of patients with encouraging results. Preliminary data suggest that RTX may favorably affect skin as well as lung disease in SSc.

Conclusions: Several basic research data underscore the potential pathogenic role of B-cells in SSc and clinical evidence suggests that RTX might be a therapeutic option in SSc. Large-scale multicenter studies are needed to evaluate the potential clinical efficacy of RTX in SSc.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Animals
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • B-Lymphocytes / immunology*
  • Disease Models, Animal
  • Fibrosis / drug therapy
  • Fibrosis / immunology
  • Fibrosis / pathology
  • Humans
  • Immunologic Factors / therapeutic use*
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / pathology
  • Lymphocyte Activation
  • Lymphocyte Depletion*
  • Male
  • Rituximab
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / pathology
  • Skin / drug effects
  • Skin / pathology


  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Rituximab