Long-term efficacy of B cell depletion therapy on lung and skin involvement in diffuse systemic sclerosis

Semin Arthritis Rheum. 2015 Feb;44(4):428-36. doi: 10.1016/j.semarthrit.2014.09.002. Epub 2014 Sep 8.


Objectives: To assess the long-term efficacy and safety of single and multiple courses of rituximab therapy in systemic sclerosis (SSc) patients with and without lung disease.

Methods: A total of 20 SSc patients with a diffuse disease were treated with rituximab. At baseline and during follow-up the lung involvement was evaluated with pulmonary function tests (FVC and DLCO) and with lung high-resolution computed tomography (HRCT).

Results: The skin score, activity, and severity indices improved significantly after 12 months and at final follow-up compared to baseline. After 12 months, there was a significant increase of FVC and TLC compared to baseline (p = 0.024 and p = 0.005, respectively), while the mean DLCO value remained stable. Considering the last available follow-up in six patients with restrictive lung disease at baseline, two patients (33.3%) experienced an increase of more than 10% of FVC, one patient had a decrease of FVC >10%, while in three patients FVC remained stable (50%). After the mean follow-up of 48.5 ± 20.4 months, among the patients with normal lung parameters at baseline, FVC remained stable in 12 (85.7%) and in one patient (14.3%) it increased by more than 10%. At the final follow-up, the alveolar and interstitial HRCT scores remained stable in more than 80% of patients, both in patients with and without restrictive lung disease at baseline.

Conclusions: Anti-CD20 B cell depletion therapy is effective on skin involvement but seems also to preserve the pulmonary function, as supported by a stable or improved FVC and stable interstitial score, suggesting a possible role of rituximab as a modifying therapy overall in early diffuse SSc.

Keywords: B cells; CD20 depletion therapy; Lung involvement; Skin fibrosis; Systemic sclerosis.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Murine-Derived / pharmacology
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Antigens, CD20 / drug effects
  • Antigens, CD20 / immunology*
  • B-Lymphocytes / drug effects
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology*
  • Cyclophosphamide / pharmacology
  • Cyclophosphamide / therapeutic use
  • Female
  • Follow-Up Studies
  • Humans
  • Lung / drug effects
  • Lung / pathology*
  • Lung / physiopathology
  • Male
  • Middle Aged
  • Respiratory Function Tests
  • Rituximab
  • Scleroderma, Systemic / diagnostic imaging
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / physiopathology
  • Severity of Illness Index
  • Skin / drug effects
  • Skin / pathology*
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Antibodies, Monoclonal, Murine-Derived
  • Antigens, CD20
  • Rituximab
  • Cyclophosphamide