Still, there is “no agreement on who to treat,” she said.
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When the time does come to treat, there is some guidance. A recent phase 2 trial with tocilizumab enrolled patients with mostly mild to moderate disease, and it showed less worsening of lung function than placebo.2
A single-center study in Greece found that treatment with rituximab resulted in better lung function (i.e., total lung capacity and diffusing capacity) and skin scores compared with placebo.3 But Dr. Pope added that not all published case series have shown a benefit from rituximab in these patients, so it is “not our home run.”
A study published earlier this year found that mycophenolate mofetil was not inferior to cyclophosphamide in SSc-ILD patients.4 And the IPF treatments nintedanib, pomalidomide and perfenidone are now being closely assessed in ILD associated with scleroderma, Dr. Pope said.5,6,7
“We don’t know about the IPF treatments,” she said. “They’re being trialed for safety. … Now they’re looking at some of them for efficacy.”
Thomas R. Collins is a freelance writer living in South Florida.
- Fernández-Codina A, Walker K, Pope J, et al. Real-life treatment strategies for systemic sclerosis according to experts. Annals of the Rheumatic Diseases. 2017;76:629.
- Khanna D, Denton CP, Jahreis A, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): A phase 2, randomised, controlled trial. Lancet. 2016 Jun 25;387(10038):2630–2640.
- Daoussis D, Liossis SN, Tsamandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012 Mar–Apr;30(2 Suppl 71):S17–S22.
- 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.
- Hsu VM, Denton CP, Domsic RT, et al. Pomalidomide in patients with interstitial lung disease due to systemic sclerosis: A phase II, multicenter, randomized, double-blind, placebo-controlled, parallel-group study. J Rheumatol. 2018 Mar;45(3):405–410.
- Distler O, Brown KK, Distler JHW, et al. Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS™). Clin Exp Rheumatol. 2017 Sep–Oct;35 Suppl 106(4):75–81. Epub 2017 Jun 29.
- Khanna D, Albera C, Fischer A, et al. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: The LOTUSS trial. J Rheumatol. 2016 Sep;43(9):1672–1679.