Recent advances in the management of systemic sclerosis-associated interstitial lung disease

Abstract

Purpose of reviewInterstitial lung disease associated with systemic sclerosis (SSc-ILD) is a frequent organ manifestation leading to high morbidity and mortality. In 2020, the European management recommendations for SSc-ILD were published. Despite being comprehensive, several questions could not be answered or no consensus was reached.Recent findingsWe highlight recent advances in the screening and early diagnosis, including surveys emphasizing that still 30–40% of all experts do not order baseline HRCTs in their SSc patients. We discuss recent advances in the assessment of disease progression, risk prediction and monitoring of SSc-ILD including novel insights in the disease course of SSc-ILD, clinical predictive factors for disease progression, the role of increasing extent of ILD on serial HRCT and radiomics, PET/CT and home spirometry as sensitive future tools to monitor SSc-ILD patients. We describe recent advances in the treatment of SSc-ILD, including novel data and trials as well as post hoc analyses of clinical trials on mycophenolate, cyclophosmphamide, tocilizumab, rituximab, riociguat and nintedanib. Lastly, we elucidate on peripheral blood cell gene expression profiling as a novel way to identify patients with a better treatment response to mycophenolate.SummaryIn this review, we highlight recent advances in the management of SSc-ILD.

Die interstitielle Lungenerkrankung bei systemischer Sklerose

“…Zu berücksichtigen sind die Charakteristika der Studienpopulation: kurze Krankheitsdauer, progressive Erkrankung, erhöhte humorale Entzündungsaktivität. Mit dem stetig wachsenden Verständnis der zugrundeliegenden Pathophysiologie wächst auch die Zahl der klinischen Phase-II/Phase-III-Studien für SSc-ILD (Review in [73]).…”

Advances in the Treatment of Systemic Sclerosis

“…Although SSc-related ILD continues to be associated with a high mortality, over the past 15 years there have been advances in treatment that can slow progression. 66 Recent review articles on SSc-related ILD provide more detail, [67][68][69][70] but key findings relevant to changing clinical practice are summarized here. Two randomized placebo-controlled trials reported in 2006 suggested benefit from cyclophosphamide.…”