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Outcomes of systemic sclerosis associated interstitial lung disease patients with a persistent inflammatory phenotype based on serial CRP measurements
  1. Elizabeth R Volkmann,
  2. Donald P Tashkin
  1. Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA
  1. Correspondence to Dr Elizabeth R Volkmann, University of California Los Angeles, Los Angeles CA 90095, California, USA; evolkmann{at}

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Interstitial lung disease (ILD) is a serious complication of systemic sclerosis (SSc) with a variable clinical trajectory.1 Both clinical and biological factors can provide prognostic information on the risk of progression of ILD; however, prognostic biomarkers do not consistently predict outcomes in patients receiving different treatments for SSc-ILD.2 In the present issue of Thorax, Guler et al demonstrate that persistent elevation in C reactive protein (CRP) is associated with an increased risk of mortality in 1171 patients with SSc-ILD.3 Notably, in this observational study, treatment with immunosuppressive therapy (defined as use of cyclophosphamide, mycophenolate mofetil, methotrexate, azathioprine, rituximab or prednisone >10 mg per day at any visit during the follow-up period) in patients with SSc-ILD with a persistent inflammatory phenotype (defined as CRP values≥5 mg/L at ≥80% of visits;) was associated with stabilisation of the course of forced vital capacity (FVC)% predicted; whereas, lack of treatment with immunosuppressive therapies was associated with a decline in FVC% predicted in this subgroup. These important findings are consistent with the results of prior randomised controlled trials in SSc-ILD demonstrating improvement or stabilisation of the FVC course in patients receiving various immunosuppressive therapies, including mycophenolate,4 cyclophosphamide,4 5 rituximab6 7 and tocilizumab.8

The study of Guler et al also found that SSc patients with a persistent inflammatory phenotype were more …

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  • Contributors ERV drafted the manuscript. DPT revised the manuscript. ERV and DPT approved the final manuscript.

  • Funding This study was funded by NHLBI Division of Intramural Research (K23 HL150237).

  • Competing interests ERV reports the following financial relationships outside of the submitted work: Consulting fees from Boehringer Ingelheim, CSL Behring, GSK; Speaking fees (unbranded disease state lectures) from Boehringer Ingelheim; Grant support from Kadmon, Forbius, Corbus, Horizon Therapeutics, Prometheus, Boehringer Ingelheim. DPT reports receiving modest financial support from Genentech for participation in an investigator-initiated trial outside of the submitted work.

  • Provenance and peer review Commissioned; internally peer reviewed.

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