系统性硬化症相关间质性肺病的诊治现状和进展

doi:10.35541/cjd.20180838

中华皮肤科杂志 ›› 2020, Vol. 53 ›› Issue (1): 66-71.doi: 10.35541/cjd.20180838

系统性硬化症相关间质性肺病的诊治现状和进展

陈冰琳薛美娟杨骥李明

复旦大学附属中山医院皮肤科，上海 200032

收稿日期:2018-10-23 修回日期:2019-04-17 发布日期:2019-12-31
通讯作者: 杨骥 E-mail:yang.ji@zs-hospital.sh.cn
基金资助:
国家自然科学基金（81472874、81573043）

Diagnosis and treatment of systemic sclerosis-associated interstitial lung disease: current status and advances

Chen Binglin, Xue Meijuan, Yang Ji, Li Ming

Department of Dermatology, Zhongshan Hospital, Fudan University, Shanghai 200032, China

Received:2018-10-23 Revised:2019-04-17 Published:2019-12-31
Contact: Yang Ji E-mail:yang.ji@zs-hospital.sh.cn
Supported by:
National Natural Science Foundation of China（81472874, 81573043）

摘要/Abstract

摘要： 【摘要】系统性硬化症（SSc）是一种罕见的伴有内脏器官纤维化的慢性结缔组织病，其并发的间质性肺病（ILD）是SSc患者死亡的首要原因。SSc相关ILD发病隐匿，不及时治疗进展迅速，患者生存质量和生存率明显下降。本文综述SSc-ILD早期诊断方法和治疗时机，并对传统的免疫抑制剂和新近出现的靶向药物、造血干细胞移植、肺移植等治疗方法做出综述。

关键词: 硬皮病, 系统性；肺疾病, 间质性；诊断；临床方案

Abstract: 【Abstract】 Systemic sclerosis （SSc） is a rare, chronic connective tissue disease with internal organ fibrosis, and interstitial lung disease （ILD） is the leading cause of death in patients with SSc. The onset of SSc-associated ILD is usually latent, and delayed treatment may lead to rapid progression, and markedly decrease the quality of life and survival rate of patients. This review summarizes approaches to the early diagnosis of SSc-associated ILD and the time-to-treatment, and provides an overview of its treatment, including traditional immunosuppressive agents, newly emerging targeted therapies, hematopoietic stem cell transplantation, lung transplantation, and so on.

Key words: Scleroderma, systemic, Lung diseases, interstitial, Diagnosis, Clinical protocols

陈冰琳薛美娟杨骥李明. 系统性硬化症相关间质性肺病的诊治现状和进展[J]. 中华皮肤科杂志, 2020,53(1):66-71. doi:10.35541/cjd.20180838

Chen Binglin, Xue Meijuan, Yang Ji, Li Ming. Diagnosis and treatment of systemic sclerosis-associated interstitial lung disease: current status and advances[J]. Chinese Journal of Dermatology, 2020, 53(1): 66-71.doi:10.35541/cjd.20180838

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系统性硬化症相关间质性肺病的诊治现状和进展

Diagnosis and treatment of systemic sclerosis-associated interstitial lung disease: current status and advances

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