硬皮病中的间质性肺疾病

Schoenfeld Sara R, Castelino Flavia V

Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.

Rheum Dis Clin North Am. 2015 May;41(2):237-48. doi: 10.1016/j.rdc.2014.12.005. Epub 2015 Feb 26.

Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.

系统性硬化症是一种病因不明的异质性疾病，有效治疗方法有限。它的特征是自身免疫、血管病变和纤维化，临床表现为多器官受累。间质性肺病是系统性硬化症的常见并发症，与显著的发病率和死亡率相关。间质性肺病的诊断取决于仔细的临床评估、肺功能测试和高分辨率计算机断层扫描。有效的治疗选择仍然有限。目前有几种实验性疗法正处于早期临床试验阶段，显示出前景。