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Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Autoimmune rheumatic diseases frequently involve the lungs, leading to severe interstitial lung disease (ILD).
  • Systemic sclerosis (SSc)-ILD is common, but ILD also occurs in rheumatoid arthritis, Sjögren's syndrome, lupus, myositis, antisynthetase syndrome, and ANCA-associated vasculitis.
  • Rituximab (RTX), an anti-CD20 B cell depleting antibody, is used for autoimmune diseases.

Purpose of the Study:

  • To review the efficacy of Rituximab (RTX) in treating interstitial lung disease (ILD) associated with autoimmune rheumatic diseases.
  • To evaluate RTX's impact on pulmonary function tests (PFTs) and chest imaging in CTD-ILD.
  • To assess RTX as a potential treatment for refractory connective tissue disease-ILD.

Main Methods:

  • Review of retrospective uncontrolled studies and some prospective studies on RTX for CTD-ILD.
  • Analysis of controlled and uncontrolled studies specifically in Systemic Sclerosis-ILD (SSc-ILD).
  • Inclusion of data on RTX use in ANCA-associated vasculitis-ILD (AAV-ILD), though data is scarce.

Main Results:

  • RTX demonstrates effectiveness in improving PFTs and chest CT findings in CTD-ILD.
  • Studies in SSc-ILD show encouraging results with B cell depletion therapy.
  • Comparative studies suggest RTX may be superior to other treatments for CTD-ILD.

Conclusions:

  • Rituximab (RTX) is a potentially effective and safe, though off-label, treatment option for refractory connective tissue disease-associated interstitial lung disease (CTD-ILD).
  • Further large-scale controlled trials are needed to confirm RTX's role in various autoimmune-ILDs.
  • RTX offers a promising alternative for patients with severe ILD unresponsive to other therapies.