小结
定义
病史和体格检查
关键诊断因素
- 存在的危险因素
- 难以进行运动作业
- 肌无力
- 肌萎缩
- 向阳性皮疹，伴有眼睑水肿
- Gottron 丘疹
其他诊断因素
- 频繁跌倒
- 疲乏和全身不适
- 体重减轻
- 呼吸短促
- 轻度发热
- 呼吸音异常
- 吞咽困难
- 肌痛
- 关节痛
- 心悸
- 晕厥
- 面部皮疹
- 红斑性皮疹
- 甲襞变化
- 面部肌无力
- 皮肤钙质沉着
- 关节肿胀
- 心律失常
- 心力衰竭和/或心肌梗死的体征
- 体检发现恶性肿瘤
- 自身免疫性疾病的全身症状。
- 周围神经病
危险因素
- 年龄>40 岁
- 高强度的全球紫外线辐射接触
- 遗传易感性
- 女性及/或黑人种族（多发性肌炎与皮肌炎）
- 男性及/或白人种族（包涵体肌炎）
- 降脂药物
- HIV
- 病毒感染（不包括 HIV）
- 非病毒感染
- 疫苗接种
- D-青霉胺
- 其他药物或毒素
诊断性检查
首要检查
- 肌酸激酶
- 肌电图
- 肌肉活检
- 肌炎特异性和相关自身抗体
- 磁共振成像 (magnetic resonance imaging, MRI)
- 醛缩酶
- 乳酸脱氢酶
- 丙氨酸氨基转移酶
- 天冬氨酸氨基转移酶
- 肌红蛋白
需考虑的检查
- 红细胞沉降率
- C 反应蛋白
- 抗核抗体
- 血清肌酐
- 血清铁蛋白
- 氟代脱氧葡萄糖正电子发射计算机断层显像（FDG-PET）/CT
新兴检查
- 超声检查
治疗流程
诱导缓解：有重度肌无力或致死性并发症
诱导缓解：无重度肌无力或危及生命的并发症
维持缓解：任意严重程度
撰稿人
作者
Hector Chinoy, PhD, FRCP, BMBS, MSc, BMedSci
Professor of Rheumatology and Neuromuscular Disease
National Institute for Health Research Manchester Biomedical Research Centre
Manchester University NHS Foundation Trust
The University of Manchester
Manchester
UK
利益声明
HC has received advisory board/lecture fees from Lilly, UCB, Janssen, and Biogen; has done consultancy work for Novartis and Momenta; has received travel expenses from Abbvie, Janssen, and Eli Lilly; and has received research grants from UCB and MedImmune. He is also an author of several references cited in this topic.
James Lilleker, PhD, MBChB
Clinical Lecturer in Neurology
Centre for Musculoskeletal Research
Division of Musculoskeletal and Dermatological Sciences
The University of Manchester
Manchester
Neurology Registrar
Manchester Centre for Clinical Neuroscience
Salford Royal NHS Foundation Trust
Manchester Academic Health Sciences Centre
Salford
UK
利益声明
JL is an author of several references cited in this topic.
鸣谢
Dr Hector Chinoy and Dr James Lilleker would like to gratefully acknowledge Dr Nizar Souayah, the previous contributor to this topic.
利益声明
NS declares that he has no competing interests.
同行评议者
Sami Khella, MD
Physician
Department of Neurology
Penn Presbyterian Medical Center
University of Pennsylvania Health System
Philadelphia
PA
利益声明
SK declares that he has no competing interests.
Anthony Arnold Amato, MD
Vice-Chairman
Department of Neurology
Brigham and Women's Hospital
Harvard Medical School
Boston
MA
利益声明
AAA declares that he has no competing interests.
鉴别诊断
- 遗传性包涵体肌炎
- 眼咽肌营养不良
- 迟发型远端肌病
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