Systemic scleroderma (SSc) is an autoimmune disease characterized by excessive collagen production and progressive fibrosis. As the disease advances, vascular injury leads to fibrosis of the skin and internal organs, among which interstitial lung disease (ILD) carries the worst prognosis. Recent advances in biomarkers, imaging techniques, and innovative therapies offer hope for improving outcomes and quality of life in patients with SSc and ILD. To evaluate the usefulness of disease biomarkers and the efficacy and safety of immunomodulatory therapies in SSc-associated ILD (SSc-ILD), a literature review was conducted using the PubMed database for studies published mainly over the last 5 years. After applying inclusion criteria, 53 clinical studies were analyzed. Treating SSc-ILD remains challenging, with therapeutic strategies aiming to suppress inflammation and limit fibrosis progression. Clinical studies have demonstrated moderate to good efficacy of immunosuppressants such as cyclophosphamide (CYC) and mycophenolate mofetil (MMF), showing improvements in lung function parameters, such as forced vital capacity (FVC), and slowing disease progression. Additionally, biological agents such as nintedanib and tocilizumab have shown promising results—nintedanib in reducing the annual rate of FVC decline and tocilizumab in decreasing inflammatory biomarkers and stabilizing pulmonary function. However, despite these therapeutic advances, many studies had small sample sizes, heterogeneous patient populations, and varying inclusion criteria. Given the challenges in diagnostics and the critical need to evaluate the efficacy alongside the safety of immunomodulatory and anti-fibrotic therapies in systemic sclerosis-associated interstitial lung disease (SSc-ILD), there remains a strong demand for large, well-designed, multicenter trials with clearly defined patient cohorts to reliably assess the long-term outcomes of agents such as tocilizumab and nintedanib.
Piecuch et al. (Thu,) studied this question.