Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. We conducted a search of PubMed (National Library of Medicine) and the Web of Science Core Collection using the key words lung, pulmonary, pneumonia, pneumonitis, and alveolar and subtypes of CTD. All clinical studies from January 1, 1980, through September 1, 2018, were reviewed for descriptions of specific therapies and their efficacy or safety and were categorized as controlled interventional trials, observational prospective or retrospective cohort studies, case series (>5 patients), and case reports (<5 patients). Low-quality reports (<5 patients) before 2000, reviews, editorials, popular science papers, and letters to the editor without complete descriptions of the therapies used or their outcomes were excluded. Directed therapy for CTD-ILD is dominated by empirical use of immunosuppressive agents, with the decision to treat, treatment choice, and treatment duration limited to cases and cohort observations. Only a few higher-level controlled studies were available specifically in scleroderma-related ILD. We summarize herein for the clinician the published treatment scope and experience, highlighted clinical response, and common adverse reactions for the management of CTD-ILD.

间质性肺病（ILD）是结缔组织病（CTD）患者的常见并发症，并显着影响发病率和死亡率。病程可能从稳定或轻度进展到更严重，肺功能迅速丧失。我们使用关键词肺，肺，肺炎，肺炎和肺泡进行了PubMed（国家医学图书馆）和Web of Science核心文献检索和CTD的子类型。回顾了1980年1月1日至2018年9月1日的所有临床研究，以了解特定疗法及其疗效或安全性的描述，并归类为对照干预试验，观察性前瞻性或回顾性队列研究，病例系列（> 5例），和病例报告（<5名患者）。2000年之前的低质量报告（<5名患者），评论，社论，流行科学论文以及致编辑的信，这些信中未完整描述所用疗法或疗效。CTD-ILD的定向治疗主要是根据经验使用免疫抑制剂，治疗的决定，治疗的选择和治疗的持续时间仅限于病例和队列观察。仅有少数针对硬皮病相关ILD的高水平对照研究。