Rituximab in autoimmune connective tissue disease-associated interstitial lung disease

Rheumatology (Oxford). 2016 Jul;55(7):1318-24. doi: 10.1093/rheumatology/kew195. Epub 2016 Apr 8.


Objective: CTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response.

Methods: We conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments. Results were collated for pulmonary function and radiological outcomes before and after treatment.

Results: Twenty-four patients were treated with rituximab. Their physiological parameters had failed to improve despite other immunomodulatory agents, with a mean change in forced vital capacity (FVC) prior to therapy of - 3.3% (95% CI - 5.6, -1.1) and mean change in diffusing capacity of carbon monoxide of - 4.3% (95% CI - 7.7, -0.9). After rituximab, radiology remained stable or improved for 11 patients, while worsening was observed in 9 patients. The decline in FVC was halted following treatment, with a mean change of + 4.1% (95% CI 0.9, 7.2), while diffusing capacity of carbon monoxide was stable [mean change +2.1% (95% CI - 1.0, 5.2)]. Patients with myositis overlap or antisynthetase syndrome appeared to respond well to treatment, with four patients showing clinically significant improvement in FVC >10%.

Conclusion: Rituximab is a therapeutic option in treatment-refractory CTD-associated ILD. Some disease subgroups may respond better than others, however, more work is needed to define its role in managing these patients.

Keywords: connective tissue disease; interstitial lung disease; rituximab; treatment.

MeSH terms

  • Adult
  • Aged
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / physiopathology
  • Connective Tissue Diseases / complications*
  • Connective Tissue Diseases / drug therapy
  • Connective Tissue Diseases / physiopathology
  • Female
  • Humans
  • Immunologic Factors / therapeutic use*
  • Lung / diagnostic imaging
  • Lung / physiopathology
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Male
  • Middle Aged
  • Radiology / methods
  • Radiology / statistics & numerical data
  • Retrospective Studies
  • Rituximab / therapeutic use*
  • Treatment Outcome


  • Immunologic Factors
  • Rituximab