Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease

Rheumatology (Oxford). 2015 Aug;54(8):1420-8. doi: 10.1093/rheumatology/kev004. Epub 2015 Mar 3.


Objective: To retrospectively evaluate the efficacy and safety of rituximab (Rtx) treatment in patients with anti-synthetase syndrome (ASS) and severe interstitial lung disease (ILD).

Methods: Patients with severe ILD and >12 months follow-up post-Rtx were identified from the Oslo University Hospital ASS cohort (n = 112). Clinical data, including pulmonary function tests (PFTs), were retrospectively collected from medical reports. Extent of ILD pre-, and post-Rtx was scored on thin-section high-resolution CT (HRCT) images and expressed as a percentage of total lung volume. Muscle strength was evaluated by manual muscle testing of eight muscle groups (MMT8).

Results: Altogether, 34/112 ASS patients had received Rtx; 24/34 had severe ILD and >12 months follow-up post-Rtx (median 52 months). In these 24 patients, the median percentage of predicted forced vital capacity, forced expiratory volume in 1 s (FEV1) and diffusing capacity of the lungs for carbon monoxide (DLCO) increased by 24%, 22% and 17%, respectively, post-Rtx. Seven patients (all with disease duration <12 months and/or acute onset/exacerbation of ILD) had >30% improvement in all three PFTs. HRCT analysis showed a median 34% reduction in ILD extent post-Rtx. MMT8 score increased post-Rtx. During follow-up, 7/34 (21%) Rtx-treated ASS patients died; 6/7 deaths were related to infections. The mortality rate in the Rtx-treated group was comparable to that of the remaining ASS cohort (25/78 deceased; 32%).

Conclusion: This study, which included 24 Rtx-treated ASS patients with severe ILD, reports improved PFTs after a median 52 months follow-up post-Rtx. The best outcome was observed in patients with a disease duration <12 months and/or acute onset/exacerbation of ILD. The study indicates that Rtx could be a treatment option for selected ASS patients, but infections should be given attention.

Keywords: anti-Jo1; anti-aminoacyl tRNA synthetase; anti-synthetase syndrome; interstitial lung disease; myositis; rituximab.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Antirheumatic Agents / therapeutic use*
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Longitudinal Studies
  • Lung / diagnostic imaging
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / physiopathology
  • Male
  • Middle Aged
  • Muscle Strength / physiology
  • Myositis / complications*
  • Myositis / physiopathology
  • Respiratory Function Tests
  • Retrospective Studies
  • Rituximab
  • Severity of Illness Index
  • Tomography, X-Ray Computed
  • Treatment Outcome


  • Antibodies, Monoclonal, Murine-Derived
  • Antirheumatic Agents
  • Rituximab

Supplementary concepts

  • Antisynthetase syndrome