Successful treatment of steroid and cyclophosphamide-resistant diffuse scleroderma-associated interstitial lung disease with rituximab

Rheumatol Int. 2012 Mar;32(3):795-8. doi: 10.1007/s00296-009-1347-z. Epub 2010 Jan 8.


Scleroderma (SSc) is a multisystem disorder characterized by fibrosis and collagen deposition in the dermis, but affects multiple organ systems, leading to esophageal dysmotility, renal failure, and interstitial lung disease (ILD). ILD is common manifestation of diffuse type of SSc and may be life threatening, and require aggressive therapy with cytotoxic agents. Although high-dose steroid and cyclophosphamide are most commonly used therapy for SSc-associated ILD, the efficacy is questionable in some cases and more effective and less toxic therapies are needed. Rituximab (RTX) is a chimeric mAb against human CD20 that depletes peripheral B cells and introduced for systemic rheumatic diseases. However, there were no enough evidences for SSc-associated ILD. We report herein a case of 47-year-old female with diffuse type of SSc with steroid and cyclophosphamide-resistant ILD that was successfully treated with RTX. Thus, we suggested that RTX could be an efficacious therapeutic modality for severe, conventional treatment-resistant SSc-associated ILD.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Cyclophosphamide / therapeutic use*
  • Drug Resistance
  • Drug Substitution
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / drug therapy*
  • Middle Aged
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Rituximab
  • Scleroderma, Diffuse / complications
  • Scleroderma, Diffuse / drug therapy*
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Antibodies, Monoclonal, Murine-Derived
  • Glucocorticoids
  • Rituximab
  • Cyclophosphamide
  • Mycophenolic Acid