原发性干燥综合征相关间质性肺疾病的研究进展

Progress of interstitial lung disease related to primary Sjogren′s syndrome

原发性干燥综合征(pSS)是一种慢性系统性自身免疫性疾病,它可累及外分泌腺及其他腺外器官,肺是最常被累及的腺外器官,而间质性肺疾病(ILD)是最常见的肺受累类型。据报道pSS-ILD的患病率为3%~11%,且一旦合并ILD,则提示生活质量下降更为明显,预后更差,病死率更高。本文系统性回顾相关文献,以期进一步明确pSS-ILD的相关危险因素,以便达到早发现、早诊断、早治疗的目的,从而达到改善患者预后及生活质量的目标。

Primary Sjogren′s syndrome(pSS)is a chronic systemic autoimmune disease.It can affect exocrine glands and other extraglandular organs.Lung is the most frequently involved extraglandular organ,and interstitial lung disease(ILD)is the most common type of lung involvement.It is reported that the morbidity of pSS-ILD is about 3%-11%,and once combined with ILD,it suggests lower quality of life,worse prognosis and higher mortality.This paper reviews the relevant literatures to achieve the goal of early detection,early diagnosis and early treatment,so as to improve the prognosis and quality of life.