Is There a Role for B-cell Depletion as Therapy for Scleroderma? A Case Report and Review of the Literature

Dimitrios Daoussis, Stamatis Nick C. Liossis, Athanassios C. Tsamandas, Christina Kalogeropoulou, Alexandra Kazantzi, Panagiotis Korfiatis, Georgios Yiannopoulos, Andrew P. Andonopoulos

Research output: Contribution to journalArticlepeer-review

45 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)127-136
Number of pages10
JournalSeminars in Arthritis and Rheumatism
Issue number2
StatePublished - Oct 2010


  • B-cells
  • Fibrosis
  • ILD
  • Rituximab
  • Scleroderma
  • Systemic sclerosis
  • Therapy

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine


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