Li Min, Du Yu, Yang Xiaojuan
Chongqing Key Laboratory of Emergency Medicine, Chongqing, 400014, China.
Department of Rheumatology and Immunology, Chongqing Emergency Medical Center/Chongqing University Central Hospital, No. 1 Health 17 Road, Chongqing, 400014, China.
Rheumatol Ther. 2025 Jul 21. doi: 10.1007/s40744-025-00785-3.
Connective tissue disease-related interstitial lung disease (CTD-ILD) refers to a range of pulmonary complications arising from connective tissue disorders, characterized by alveolar inflammation and interstitial fibrosis. Various factors, such as the specific type of connective tissue disease, infections, and hypoxemia, influence acute exacerbations of CTD-ILD. Treatment strategies typically include targeted interventions for precipitating factors, glucocorticoids, immunosuppressants, and supportive care. Glucocorticoids play a key role in managing acute exacerbations of CTD-ILD; however, the optimal dosing and duration of treatment remain uncertain. Immunosuppressants show potential therapeutic value, but further studies are needed to determine the most effective regimens for CTD-ILD patients. Supportive care, including respiratory support and oxygen therapy, is crucial for symptom relief and correction of hypoxia. Despite recent treatment advancements in China, significant challenges remain in optimizing outcomes and improving survival rates in CTD-ILD, highlighting the need for a comprehensive, individualized management approach for Chinese patients. Future research should focus on elucidating the pathogenesis of CTD-ILD, tailoring treatment strategies, and establishing standardized diagnostic and management protocols based on the Chinese population.
结缔组织病相关间质性肺疾病(CTD-ILD)是指由结缔组织疾病引起的一系列肺部并发症,其特征为肺泡炎症和间质纤维化。多种因素,如结缔组织病的具体类型、感染和低氧血症,会影响CTD-ILD的急性加重。治疗策略通常包括针对诱发因素的靶向干预、糖皮质激素、免疫抑制剂和支持治疗。糖皮质激素在CTD-ILD急性加重的管理中起关键作用;然而,最佳给药剂量和治疗持续时间仍不确定。免疫抑制剂显示出潜在的治疗价值,但需要进一步研究以确定针对CTD-ILD患者的最有效方案。支持治疗,包括呼吸支持和氧疗,对于缓解症状和纠正缺氧至关重要。尽管中国近年来在治疗方面取得了进展,但在优化CTD-ILD的治疗效果和提高生存率方面仍面临重大挑战,这凸显了对中国患者采取全面、个体化管理方法的必要性。未来的研究应侧重于阐明CTD-ILD的发病机制、调整治疗策略以及基于中国人群建立标准化的诊断和管理方案。