Systemic Sclerosis-Associated Interstitial Lung Disease: Improved Understanding and Advances in Management.
Authors
Affiliations (2)
Affiliations (2)
- Department of Medicine, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
- Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Abstract
Interstitial lung disease (ILD), a common manifestation of systemic sclerosis (SSc), has the highest organ-specific morbidity and mortality, particularly in patients with diffuse cutaneous SSc (dcSSc). Recent advances in diagnostics-including artificial intelligence (AI)-enhanced high-resolution computed tomography (HRCT) and biomarkers such as Krebs van den Lungen (KL)-6-have enabled earlier detection and monitoring of disease progression. Therapeutically, the approval of antifibrotics like nintedanib (NINT) and immunomodulators such as tocilizumab (Toci) has significantly expanded treatment options. Updated international guidelines from the American College of Rheumatology (ACR), American College of Chest Physicians (CHEST), American Thoracic Society (ATS), and European league against Rheumatism (EULAR) now reflect this evolving landscape. This review aims to provide a comprehensive synthesis of screening, monitoring, and therapeutic strategies in systemic sclerosis-associated interstitial lung disease (SSc-ILD), emphasizing recent advances in AI-based imaging, serological biomarkers, and updated international treatment guidelines. We conclude by highlighting the shift toward combination and potentially triple therapy approaches, and we propose future research directions aimed at improving biomarker validation, mechanistic understanding, and personalized treatment strategies.