Interstitial lung diseases (ILDs) are frequent in systemic sclerosis (SSc) and affect approximately 50% of the patients during their disease course. SSc-ILD shows overlapping pathogenesis and clinical features with ILDs from other CTDs including SLE. In SSc, SSc-ILD is the most frequent cause of death, not only affecting patients with diffuse cutaneous SSc, but also with limited cutaneous SSc. In recent years, there have been breakthroughs in the treatment of SSc-ILD. This presentation will be based on a recent interdisciplinary consensus paper using state of the art consensus methodology to reach statements on treatments and other aspects of SSc-ILD.¹ Level of evidence for the different treatment options will be discussed. Specifically, first line treatment recommendations include cyclophosphamide, mycophenylate mofetil and nintedanib.² Second line treatments include rituximab, stem cell transplantation and early listing for lung transplantation, if appropriate. In addition, a very recent published Phase 3 trial point to efficacy of tocilizumb in patients with inflammatory, early, diffuse, recently skin-progressive SSc-ILD.³ Open questions remain about the sequence of therapy selection, combination therapy, step up or step down combination therapy and personalised medicine in a very heterogeneous patient population.

Learning Objectives

• Explain the heterogeneity of ILDs in both pathophysiology and clinical course

• Apply assessment of evidence level to clinical studies in ILDs

• Describe the new treatment options and the need for individualized treatments of SSc-ILD

• Discuss open questions and unmet needs in the ILDs

References

1. Hoffmann-Vold A-M, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheumatol 2020;2:e71–83

2. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N. Engl. J. Med. 2019;380(26):2518–28.

3. Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med 2020 Oct;8(10):963–974.