小结
定义
病史和体格检查
关键诊断因素
- 40 至 60 岁
- 发热
- 体重减轻
- 肌痛或关节痛
- 多发性单神经炎
- 感觉异常
- 肌肉压痛
- 腹痛
- 皮肤表现
- 舒张压＞90 mmHg
- 在 HBV 常规筛查前的输血史
- 既往或现在静脉吸毒
- 乙型肝炎病毒 (HBV) 的近期感染
- 睾丸痛
其他诊断因素
- 男性
- 毛细胞白血病
- 癫痫发作
- 上运动神经元无力
- 消化道出血
- 腹膜炎
- 单眼失明
- 胸痛
- 充血性心力衰竭
- 触痛性乳房肿块
危险因素
- 乙型肝炎病毒 (HBV) 感染
- 40 至 60 岁
- 毛细胞白血病
- 在 HBV 常规筛查前的输血史
- 丙型肝炎病毒 (HCV) 感染
- 男性
诊断性检查
首要检查
- C 反应蛋白 (CRP)
- 红细胞沉降率 (ESR)
- 全血细胞计数 (FBC）
- 补体
- 血清肌酐
- 中段尿分析
- 肝功能检查
- HBV 血清学检查
- 丙型肝炎病毒 (HCV) 血清学
- 冷球蛋白
- 血培养
- 肌酸激酶
- 抗中性粒细胞胞质抗体 (ANCA)
- 抗核抗体 (ANA)
- 抗双链 DNA 抗体（抗 dsDNA）
- 类风湿因子
- 抗环瓜氨酸肽（抗 CCP 抗体）
- 狼疮抗凝物
- 抗磷脂抗体免疫球蛋白 G
- B2 糖蛋白
- 纤维蛋白原
- 传统数字减影血管造影
- 超声心动图
需考虑的检查
- 磁共振血管成像 (MRA)
- CT 血管造影术
- 受累组织活检
- HIV 血清学
- 针对腺苷脱氨酶 2（ADA2）缺陷的基因检测
治疗流程
非 HBV 相关 PAN
HBV 相关 PAN
疾病复发
撰稿人
作者
Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP
Consultant Rheumatologist
Auckland and Counties Manukau District Health Boards
Auckland
New Zealand
利益声明
RS declares that he has no competing interests.
Joanna Robson, MBBS, PhD, MRCP
Rheumatology Registrar
Nuffield Orthopaedic Centre
Oxford
UK
利益声明
JR declares that she has no competing interests.
Raashid Luqmani, DM, FRCP, FRCP(E)
Professor of Rheumatology
NIHR Musculoskeletal Biomedical Research Unit
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science
University of Oxford
Oxford
UK
利益声明
RL is an author of a number of references cited in this topic.
鸣谢
Dr Ravi Suppiah, Dr Joanna Robson and Dr Raashid Luqmani would like to gratefully acknowledge Dr Loic Guillevin, the previous contributor to this topic. LG is an author of a number of references cited in this topic.
同行评议者
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.
Richard Watts, MA, DM, FRCP
Consultant Rheumatologist
Ipswich Hospital
Ipswich
UK
利益声明
RW has received fees for consulting from Roche Pharmaceuticals, manufacturer of rituximab. His department has received financial support from Wyeth Pharmaceuticals, manufacturer of etanercept, and from Schering-Plough, manufacturer of infliximab. RW is an author of a number of references cited in this topic.
Ellen C. Ebert, MD
Professor of Medicine
Department of Medicine
UMDNJ-Robert Wood Johnson Medical School
New Brunswick
NJ
利益声明
ECE declares that she has no competing interests.
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