Piecuch Dawid, Hanczyk Edyta, Zemsta Katarzyna, Zwoliński Michał, Kopciał Szymon, Jońska Joanna
Faculty of Medicine, University of Radom, 26-600 Radom, Poland.
Faculty of Medical and Health Sciences, University of Radom, 26-600 Radom, Poland.
Diagnostics (Basel). 2025 Sep 4;15(17):2243. doi: 10.3390/diagnostics15172243.
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.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征是胶原蛋白过度产生和进行性纤维化。随着疾病进展，血管损伤会导致皮肤和内脏器官纤维化，其中间质性肺病（ILD）预后最差。生物标志物、成像技术和创新疗法的最新进展为改善SSc和ILD患者的治疗效果及生活质量带来了希望。为了评估疾病生物标志物的实用性以及免疫调节疗法在SSc相关ILD（SSc-ILD）中的疗效和安全性，我们使用PubMed数据库对主要在过去5年发表的研究进行了文献综述。应用纳入标准后，分析了53项临床研究。治疗SSc-ILD仍然具有挑战性，治疗策略旨在抑制炎症并限制纤维化进展。临床研究已证明免疫抑制剂如环磷酰胺（CYC）和霉酚酸酯（MMF）具有中度至良好的疗效，显示出肺功能参数如用力肺活量（FVC）有所改善，并减缓了疾病进展。此外，尼达尼布和托珠单抗等生物制剂已显示出有前景的结果——尼达尼布可降低FVC的年下降率，托珠单抗可降低炎症生物标志物并稳定肺功能。然而，尽管有这些治疗进展，许多研究的样本量较小、患者群体异质性大且纳入标准各异。鉴于诊断方面的挑战以及迫切需要评估免疫调节和抗纤维化疗法在系统性硬化症相关间质性肺病（SSc-ILD）中的疗效和安全性，仍然强烈需要进行大型、设计良好、多中心的试验，有明确界定的患者队列，以可靠地评估托珠单抗和尼达尼布等药物的长期疗效。
