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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 …
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.