Systemic Sclerosis-Associated Interstitial Lung Disease: What We Know and How to Incorporate Guidelines Into Clinical Practice.

Although treatment options for SSc with ILD remain limited, randomized controlled trials have demonstrated the safety and efficacy of approved and unapproved therapies.

尽管治疗系统性硬化症合并间质性肺病的选项仍然有限，但随机对照试验已经证明了已批准和未批准疗法的安全性和有效性。

However, numerous unanswered questions remain regarding management of SSc with ILD, and 3 recently published clinical practice guidelines highlight the complexity of treating this condition.

然而，关于系统性硬化症合并间质性肺病（SSc with ILD）的管理，仍有许多未解之谜，最近发布的3项临床实践指南突显了治疗这种状况的复杂性。

These guidelines offer distinct and sometimes contrasting views on treatment of SSc with ILD, creating unique challenges for clinicians.

这些指南对SSc with ILD的治疗提供了不同的，有时甚至是相互矛盾的观点，为临床医生带来了独特的挑战。

The present review aimed to synthesize evidence regarding the management of SSc with ILD with a focus on how to apply the recent guidelines into practice, beginning with screening for disease and extending to the intricate decision-making surrounding treatment.

本综述旨在综合关于系统性硬化症合并间质性肺病（SSc-ILD）管理的证据，重点是如何将最新的指南应用于实践，从疾病筛查开始，延伸到治疗决策的复杂过程。

review_findings

Although an abundance of evidence supports screening all patients with SSc for ILD with high-resolution CT imaging of the chest at the time of diagnosis, emerging evidence underscores the importance of rescreening patients who possess ≥ 1 high-risk factors (male sex, Black race, advanced age, diffuse cutaneous disease, high modified Rodnan skin score, antitopoisomerase antibody positivity, nucleolar antinuclear antibodies, and impaired lung function) or if physiologic changes, new respiratory symptoms, or both attributable to ILD emerge.

尽管大量证据支持在诊断时对所有SSc患者进行胸部高分辨率CT筛查以发现ILD，但新兴的证据强调了对于具有≥1个高风险因素（男性、黑人种族、高龄、弥漫性皮肤病变、高改良Rodnan皮肤评分、抗拓扑异构酶抗体阳性、核仁型抗核抗体阳性以及肺功能受损）的患者，或者如果出现与ILD相关的生理变化、新的呼吸症状或两者均出现时，重新筛查的重要性。

Patients with established SSc with ILD should be monitored closely for the development of progressive pulmonary fibrosis.

已确诊的系统性硬化症（SSc）伴有间质性肺病（ILD）的患者应密切监测，以发现进行性肺纤维化的进展。

The guidelines all recommended, with varying degrees of conditionality, treatment with mycophenolate, tocilizumab, rituximab, cyclophosphamide, and nintedanib.

各种指南都推荐，在不同程度的条件性下，对患者进行吗替麦考酚酯、托珠单抗、利妥昔单抗、环磷酰胺和尼达尼布的治疗。

The quality of evidence was rated as low for most therapies, and the only therapy strongly recommended for SSc with ILD was mycophenolate.

大多数治疗方法的证据质量被评为低，唯一强烈推荐用于伴有间质性肺病的系统性硬化症的治疗是麦考酚酯。

summary

This review provides a comprehensive appraisal of current approaches to the diagnosis and treatment of SSc with ILD.

本综述对当前诊断和治疗伴有间质性肺病的系统性硬化症的方法进行了全面评估。

It also highlights gaps in knowledge and proposes future opportunities to develop a precision-guided approach to diagnosing and treating SSc with ILD.

它还指出了知识上的空白，并提出了未来的机会，以发展一种精确指导的方法来诊断和治疗伴有间质性肺病（ILD）的系统性硬化症（SSc）。