Among the newer treatments for ILD in SSc are nintedanib, an antifibrotic agent, and tocilizumab, an inhibitor of interleukin (IL) 6. In the SENSCIS study, 576 patients with SSc-related ILD were randomly assigned to receive 150 mg of nintedanib by mouth twice daily or placebo. The primary end point was annual rate of decline in forced vital capacity (FVC) assessed over 52 weeks. Although patients in both arms of the study saw decline in the FVC, this decrease was less in the nintedanib group compared with the placebo group.6
In the focuSSced trial, more than 200 patients with diffuse cutaneous SSc were randomly assigned to receive 162 mg of subcutaneous tocilizumab or placebo weekly for 48 weeks. In this study, the primary skin fibrosis end point was not met, but findings for the secondary end point of FVC% predicted indicated that tocilizumab may preserve lung function in people with early SSc-ILD who also have elevated acute-phase reactants.
Based on these results, the FDA approved tocilizumab for the treatment of SSc-ILD, and this medication is now part of the pantheon of treatments for patients.
In Sum
Although no panacea for treatment of the condition exists, more can be done for patients than in years past.
Jason Liebowitz, MD, is an assistant professor of medicine in the Division of Rheumatology at Columbia University Vagelos College of Physicians and Surgeons, New York.
References
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- Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: A potential clinical marker of pulmonary arterial hypertension. J Rheumatol. 2010 Jan;37(1):98–104.
- Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618–3731.
- Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006 Jun 22;354(25):2655–2666.
- Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): A randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016 Sep;4(9):708–719.
- Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019 Jun 27;380(26):2518–2528.
