a Department of Respiratory Medicine and Allergy , Tosei General Hospital , Seto , Japan.
b Department of Medicine, Divisions of Rheumatology, Pulmonary Sciences and Critical Care Medicine , University of Colorado , Aurora , CO , USA.
Expert Rev Respir Med. 2017 Jul;11(7):591-603. doi: 10.1080/17476348.2017.1335600. Epub 2017 Jun 2.
Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD). Although the majority of patients with CTD-ILD are stable or slowly progressive, a significant group exhibits a more severe and progressive decline. Interstitial pneumonia with autoimmune features (IPAF) describes the subset of patients with interstitial pneumonia who have features suggesting underlying autoimmunity, but whose features fall short of a clear diagnosis of CTD. Areas covered: In this focused review, we discuss recent advances in early detection, prognostic evaluation, and management of autoimmune forms of ILD. Expert commentary: Early detection of ILD and a better understanding of factors that impact prognostication may be helpful when making decisions regarding therapeutic interventions. The treatment of CTD-ILD should be comprehensive, is often fraught with challenges and can be complicated by comorbid conditions and extra-thoracic disease activities. Several large randomized studies have examined the impact of immunosuppressive therapy for CTD-ILD, however, additional studies are needed to determine the optimal treatment strategies. Future studies may provide additional information about the best treatments in patients with IPAF.
间质性肺病（ILD）是结缔组织病（CTD）的常见表现。虽然大多数 CTD-ILD 患者病情稳定或进展缓慢，但有相当一部分患者病情更为严重且进展迅速。自身免疫特征性间质性肺炎（IPAF）描述的是一组具有间质性肺炎特征且提示潜在自身免疫的患者，但这些特征尚未达到明确的 CTD 诊断标准。
在本次重点综述中，我们讨论了自身免疫性 ILD 的早期检测、预后评估和管理方面的最新进展。
ILD 的早期发现以及更好地了解影响预后的因素，在决定治疗干预措施时可能会有所帮助。CTD-ILD 的治疗应全面，往往充满挑战，并且可能会因合并症和胸外疾病活动而变得复杂。几项大型随机研究已经研究了免疫抑制疗法对 CTD-ILD 的影响，然而，还需要进一步的研究来确定最佳的治疗策略。未来的研究可能会为 IPAF 患者的最佳治疗方法提供更多信息。
