系统性硬化症（硬皮病）
小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 雷诺现象
- 手指凹陷或溃疡
- 手足肿胀
- 皮肤增厚
- 手部功能丧失
- 指端硬化
- 烧心、反酸和吞咽困难
- 腹胀
- 大便失禁
- 关节痛和肌痛
- 甲襞毛细血管异常
- 毛细血管扩张
- 皮下钙质沉着
- 呼吸困难
- 肺底存在干湿罗音
- 肌腱摩擦音
- 突发中度/重度高血压
其他诊断因素
- 疲劳
- 干咳
- 运动耐量下降
- 体重减轻
- 炎症性关节炎
- 近端肌肉虚弱（炎性肌病）
- 滑膜炎
- 肺动脉瓣区第二心音 (S2) 增强加剧
- 贫血征象
危险因素
- 硬皮病家族史
- 免疫失调（例如 ANA 阳性）
- 暴露于环境物质和毒素（例如硅尘或溶剂）
诊断性检查
首要检查
- 血清自身抗体
- 全血细胞计数 (FBC）
- 尿素和血清肌酐
- 红细胞沉降率 (ESR)
- C 反应蛋白 (CRP)
- 尿显微镜检查
- 全套肺功能检查（肺量测定、肺容量和弥散量测定）
- 心电图 (ECG)
- 超声心动图
- 胸部 X 线检查
- 钡餐
需考虑的检查
- 高分辨率胸部 CT 扫描
- 上消化道内镜 ± 活检
- 血清肌酶
- 肌电图/神经传导检查
- 肌肉活检
治疗流程
硬皮病肾危象
心脏填塞
雷诺现象：无溃疡
出现雷诺现象：伴有手指溃疡
全身性皮肤受累
胃肠受累
肌病
滑膜炎、肌腱摩擦音或炎症性关节炎
间质性肺病
心包积液
肺动脉高压
撰稿人
作者
Maureen D. Mayes, MD, MPH
Professor of Medicine
Division of Rheumatology and Clinical Immunogenetics
University of Texas Health Science Center
Houston Medical School
Houston
TX
利益声明
MDM is an advisory board member for Boehringer-Ingelheim, Galapagos, Corbus, and Eicos. MDM is on the grant review board for Medtelligence. She receives royalties from Oxford University Press and Henry Stewart Talks. MDM is an author of a number of references cited in this topic.
鸣谢
Dr Maureen D. Mayes would like to gratefully acknowledge Dr Rajpreet K. Arora-Singh, a previous contributor to this topic. RKA declares that she has no competing interests.
同行评议者
Mary Ellen Csuka, MD
Associate Professor of Medicine
Department of Rheumatology
Medical College of Wisconsin
Milwaukee
WI
利益声明
MEC has been reimbursed by: Actelion International (and is a member of its speakers bureau); Encysive Pharmaceuticals; and MediQuest Therapeutics. She is local principal investigator for the SCOT trial, sponsored by NIH.
Jerry Molitor, MD, PhD
Associate Professor
Division of Rheumatic and Autoimmune Diseases
Department of Medicine
University of Minnesota
Minneapolis
MN
利益声明
JM declares that he has no competing interests.
Bridget Griffiths, MD, FRCP(UK)
Consultant Rheumatologist
Department of Rheumatology
Freeman Hospital
Newcastle Upon Tyne
UK
利益声明
BG declares that she has no competing interests.
Ariane L. Herrick, MD, FRCP
Reader in Rheumatology and Consultant Rheumatologist
Musculoskeletal Research Group
University of Manchester
Manchester
UK
利益声明
ALH declares that she has no competing interests.
鉴别诊断
- 原发性雷诺现象
- 局限性硬皮病（硬斑病）
- 混合结缔组织病
更多 鉴别诊断指南
- 治疗系统性硬化的 EULAR 建议更新。
- 2015 年欧洲心脏病学会/欧洲呼吸学会(ESC/ERS) 肺动脉高压诊断和治疗指南
更多 指南
登录或订阅即可浏览 BMJ Best Practice 临床实践完整内容
内容使用需遵循免责声明