最近审阅: 6 十一月 2020
最近更新: 29 三月 2018

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(肺活量、肺容量和弥漫量测定)
  • 心电图 (ECG)
  • 超声心动图
  • 胸部 X 线检查 (CXR)
  • 钡餐
更多 首要检查

需考虑的检查

  • 高分辨率胸部 CT 扫描
  • 上消化道内镜检查 ± 活检
  • 血清肌酶
  • EMG /神经传导检查
  • 肌肉活检
更多 需考虑的检查

治疗流程

撰稿人

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, Genentech and Astellas. She receives royalties from Oxford University Press for "The Scleroderma Book: A guide for patients and families". MDM is an author of a number of references cited in this monograph.

Dr Maureen D. Mayes would like to gratefully acknowledge Dr Rajpreet K. Arora-Singh, a previous contributor to this monograph. RKA declares that she has no competing interests.

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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.

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.

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle Upon Tyne

UK

利益声明

BG declares that she has no competing interests.

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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