硬皮病中的间质性肺疾病
Interstitial lung disease in scleroderma.
作者信息
Schoenfeld Sara R, Castelino Flavia V
机构信息
Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
出版信息
Rheum Dis Clin North Am. 2015 May;41(2):237-48. doi: 10.1016/j.rdc.2014.12.005. Epub 2015 Feb 26.
Abstract
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.
摘要
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。
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