Chung Sang Wan
Division of Rheumatology, Department of Internal Medicine, Kyung Hee University College of Medicine, Kyung Hee University Hospital, Seoul 02447, Republic of Korea.
Biomedicines. 2025 Jun 13;13(6):1463. doi: 10.3390/biomedicines13061463.
Progressive fibrosing interstitial lung disease (PF-ILD) is a significant complication of connective tissue diseases (CTDs), particularly in systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathies (IIM). Despite clinical similarities with idiopathic pulmonary fibrosis (IPF), CTD-associated ILDs exhibit distinct pathogenetic and immunologic features. This review aims to summarize key predictive biomarkers and current treatment strategies associated with the progressive fibrosing phenotype in SSc-ILD, RA-ILD, and IIM-ILD. We conducted a focused literature search of PubMed and Scopus databases covering publications from January 2010 to February 2024. Included studies evaluated serum, cellular, or genetic biomarkers with predictive value for disease progression or treatment response. Only peer-reviewed English-language articles were included. Exclusion criteria encompassed single case reports and editorials. Several biomarkers, including KL-6, SP-D, CXCL4, and anti-MDA5, demonstrate potential in predicting fibrotic progression in CTD-ILDs. However, variability in sensitivity and specificity across CTD subtypes limits broad clinical applicability. Therapeutic agents such as nintedanib and pirfenidone show efficacy in slowing lung function decline. Biologics including rituximab and tocilizumab offer additional options, particularly in immunologically active diseases. Although promising biomarkers and therapies are emerging, no single marker or intervention currently predicts or modifies PF-ILD outcomes across all CTD subsets. Prospective studies and integrative biomarker panels are needed to improve patient stratification and guide therapy.
进行性纤维化间质性肺疾病（PF-ILD）是结缔组织病（CTD）的一种重要并发症，尤其是在系统性硬化症（SSc）、类风湿关节炎（RA）和特发性炎性肌病（IIM）中。尽管与特发性肺纤维化（IPF）在临床上有相似之处，但CTD相关的间质性肺疾病表现出不同的发病机制和免疫特征。本综述旨在总结与SSc-ILD、RA-ILD和IIM-ILD中进行性纤维化表型相关的关键预测生物标志物和当前治疗策略。我们对PubMed和Scopus数据库进行了针对性的文献检索，涵盖2010年1月至2024年2月的出版物。纳入的研究评估了对疾病进展或治疗反应具有预测价值的血清、细胞或遗传生物标志物。仅纳入经同行评审的英文文章。排除标准包括单病例报告和社论。几种生物标志物，包括KL-6、SP-D、CXCL4和抗MDA5，在预测CTD-ILDs的纤维化进展方面显示出潜力。然而，不同CTD亚型的敏感性和特异性存在差异，限制了其广泛的临床应用。诸如尼达尼布和吡非尼酮等治疗药物在减缓肺功能下降方面显示出疗效。包括利妥昔单抗和托珠单抗在内的生物制剂提供了额外的选择，特别是在免疫活性疾病中。尽管有前景的生物标志物和疗法不断涌现，但目前尚无单一标志物或干预措施能够预测或改变所有CTD亚组的PF-ILD结局。需要进行前瞻性研究和综合生物标志物 panel 来改善患者分层并指导治疗。
