最后审阅: 18 九月 2021
最后更新: 25 八月 2020

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 (CXR)
  • 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

撰稿人

作者

Maureen D. Mayes, MD, MPH
Maureen D. Mayes

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.

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