Identification and management of interstitial lung disease associated with systemic sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), and polymyositis/dermatomyositis (PM/DM-ILD): development of expert consensus-based clinical algorithms

Clinical guidance on the identification and management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is needed for optimal clinical practice. We aimed to develop clinical algorithms for identifying and managing three common CTD-ILDs: those associated with systemic sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), and polymyositis/dermatomyositis (PM/DM-ILD). Meetings were held October – November 2023 to create consensus-based algorithms for identifying and managing SSc-ILD, RA-ILD, and PM/DM-ILD in clinical practice, based on expert consensus statements for identification and management of CTD-ILD previously derived from a Delphi process. We developed clinical algorithms for SSc-ILD, RA-ILD, and PM/DM-ILD that highlight both commonalities and differences in the identification and management of these CTD-ILDs. Importantly, ILD should be suspected in patients with SSc, RA, or PM/DM who have respiratory symptoms. Chest high-resolution computed tomography has utility for screening, diagnosis and assessment of severity. Furthermore, regular follow-up and multidisciplinary management are important. Disease-specific considerations include unique risk factors such as anti-topoisomerase I antibodies in SSc-ILD, high-titer cyclic citrullinated peptide antibodies in RA, anti-aminoacyl tRNA synthetase antibodies in PM/DM, and anti-melanoma differentiation-associated gene 5 antibody in DM. These algorithms may help physicians to identify and manage patients with SSc-ILD, RA-ILD, or PM/DM-ILD.

结缔组织病相关间质性肺疾病（connective tissue disease-associated interstitial lung disease, CTD-ILD）的识别与诊疗临床指南，是优化临床实践的必需内容。本研究旨在针对三类常见CTD-ILD制定临床诊疗算法：分别为与系统性硬化症（systemic sclerosis, SSc）相关的SSc-ILD、与类风湿关节炎（rheumatoid arthritis, RA）相关的RA-ILD，以及与多发性肌炎/皮肌炎（polymyositis/dermatomyositis, PM/DM）相关的PM/DM-ILD。研究团队于2023年10月至11月召开会议，基于此前通过德尔菲法（Delphi process）制定的CTD-ILD识别与诊疗专家共识声明，构建面向临床实践的SSc-ILD、RA-ILD及PM/DM-ILD诊疗共识算法。本研究制定的SSc-ILD、RA-ILD及PM/DM-ILD临床算法，凸显了此类CTD-ILD在识别与诊疗环节中的共性与差异。值得注意的是，合并呼吸系统症状的SSc、RA或PM/DM患者，均应怀疑合并ILD。胸部高分辨率计算机断层扫描（high-resolution computed tomography）可用于筛查、诊断及病情严重程度评估。此外，定期随访与多学科诊疗同样至关重要。不同疾病的特异性考量包括特有危险因素：如SSc-ILD相关的抗拓扑异构酶I抗体（anti-topoisomerase I antibodies）、RA相关的高滴度抗环瓜氨酸肽抗体（high-titer cyclic citrullinated peptide antibodies）、PM/DM相关的抗氨酰tRNA合成酶抗体（anti-aminoacyl tRNA synthetase antibodies），以及皮肌炎相关的抗黑色素瘤分化相关基因5抗体（anti-melanoma differentiation-associated gene 5 antibody）。上述临床算法可辅助临床医师识别并诊疗SSc-ILD、RA-ILD或PM/DM-ILD患者。