小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 手指苍白/疼痛
- 关节炎/关节痛
- 手部肿胀
- 指端硬化
- 甲襞血管变化
- 呼吸困难或咳嗽
- GORD 和烧心症状
- 肌痛或肌炎
其他诊断因素
- 血尿
- 淋巴结肿大
- 脱发
- 皮疹
- 近端肌肉无力
- 三叉神经痛
- 头痛
- 神经精神性疾病，包括精神病和癫痫发作
- 周围神经病
- 发热
危险因素
- 女性
- 年龄为 20 至 40 岁
- 存在特异性 HLA 基因型
诊断性检查
首要检查
- 全血细胞计数 (FBC）
- 红细胞沉降率
- C 反应蛋白 (CRP)
- 血清尿素和肌酐
- 类风湿因子
- 抗核抗体
- 抗环瓜氨酸肽（抗 CCP）抗体
- 尿液分析
- 抗 U1 核糖核蛋白
- 抗 Jo-1
需考虑的检查
- 肌酸激酶
- 抗双链 DNA
- Smith 抗原
- 抗 SS-A 和抗 SS-B
- 其他抗体检测（抗-Scl 70 抗体、抗着丝点抗体、抗-RNA 聚合酶 Ⅲ 抗体、抗-PM/Scl 抗体）
- 肺功能试验(肺活量，肺容量，肺弥散功能测定)
- 胸部 X 线检查 (CXR)
- 高分辨率胸部 CT 扫描
- 超声心动图
- 右心导管术
- 钡餐
- 上消化道内镜 ± 活检
- 受累关节普通 X 线片
- 肌电图
- 神经传导检查
- 肌肉活检
- 肺活组织检查
- 肾脏活检
新兴检查
- 抗 Ku 抗体
- 其他抗合成酶抗体（PL7、PL12、OJ、EJ、KS、Ha 等）
治疗流程
所有患者
撰稿人
作者
Larry Young, MD
Associate Professor of Medicine
Division of Rheumatology
Miller School of Medicine
University of Miami
Miami
FL
利益声明
LY declares that he has no competing interests.
Carlos Lozada, MD
Associate Professor of Medicine
Division of Rheumatology and Immunology
Miller School of Medicine
University of Miami
Miami
FL
利益声明
CL declares that he has no competing interests.
鸣谢
Dr Larry Young and Dr Carlos Lozada would like to gratefully acknowledge Dr Robert Hoffman, a previous contributor to this topic.
利益声明
RH has received NIH grants greater than 6 figures USD. RH is the author of some studies referenced in this topic. RH declares that he has no competing interests.
同行评议者
Robert Ortmann, MD
Associate Professor and Director
Division of Rheumatology and Immunology
University of Arkansas for Medical Sciences
Little Rock
AR
利益声明
RO declares that he has no competing interests.
Alan Bridges, MD
Professor and Vice Chair
Department of Medicine
University of Wisconsin Hospital
Madison
WI
利益声明
AB declares that he has no competing interests.
Simon Bowman, PhD, FRCP
Consultant Rheumatologist and Honorary Senior Clinical Lecturer in Rheumatology
Selly Oak Hospital
Birmingham
UK
利益声明
SB declares that he has no competing interests.
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