小结
定义
病史和体格检查
关键诊断因素
- 哮喘、变应性鼻炎或鼻窦炎病史
- 局灶性麻木或无力
- 鼻腔渗出、鼻塞或面部痛
- 高出皮面的紫癜及瘀点
- 喘鸣
- 咯血
- 皮肤结节
其他诊断因素
- 与用药的关系
- 疲劳、关节痛、肌肉痛
- 气短或咳嗽
- 腹痛
- 感觉或运动缺失
- 呼吸急促
- 啰音
- 外周水肿
- 端坐呼吸
- 肝颈静脉回流征
危险因素
- 哮喘、变应性鼻炎或鼻窦炎病史
- 与用药的关系
诊断性检查
首要检查
- 全血细胞计数 (FBC) 和分类计数
- 血清抗中性粒细胞胞浆抗体（ANCA）
- 血清 CRP
- 红细胞沉降率
- 血清尿素和肌酐
- 尿液分析
- 肺功能试验
- 胸部 X 线检查
- 超声心动图
需考虑的检查
- 流式细胞仪检测外周血
- FIP1L1/PDGFR alpha突变的分子检测
- 血清 IgE 水平
- 烟曲霉血清特异性 IgE 和 IgG
- 大便培养以寻找寄生虫及虫卵
- 弓蛔虫血清学检查
- 胸部 CT
- 肌电图
- 支气管镜及支气管肺泡灌洗（BAL）
- 受累组织活检
- 下肢超声
- CT 血管造影术
- 骨髓活组织检查
- 巯基嘌呤甲基转移酶水平
- HIV 检测
新兴检查
- 心脏磁共振成像
- 嗜酸粒细胞活化趋化因子-3
治疗流程
非重度 EGPA
重度 EGPA
成功缓解后复发
撰稿人
作者
Karina Keogh, MBBCh
Assistant Professor
Division of Pulmonary and Critical Care Medicine
Mayo Clinic
Rochester
MN
利益声明
KK is an author of a number of references cited in this topic and has developed educational material on Churg-Strauss syndrome for First Consult (Elsevier). KK has undertaken contractual research for GlaxoSmithKline (utilizing mepolizumab in asthma trials) and AstraZeneca (asthma trial), but received no personal financial gain for this work.
鸣谢
Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this topic.
利益声明
GK is an author of some references cited in this topic.
同行评议者
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 has organisational financial interests with funds received for consultation and training on disease assessment in vasculitis for GlaxoSmithKline, Nordic, and Chemocentryx. RL has received personal funds for lectures on vasculitis from Roche and Nippon Kayaku. RL is an author of a reference cited in this topic.
Matthew D. Morgan, MB ChB, MRCP, PhD
Clinical Senior Lecturer in Renal Medicine
Centre for Translational Inflammation Research
University of Birmingham Research Laboratories
Queen Elizabeth Hospital Birmingham
Birmingham
UK
利益声明
MDM has been a paid consultant for Sphere Medical Ltd, and has received research grants and funding from GlaxoSmithKline and Novartis.
Philip Seo, MD, MHS
Assistant Professor of Medicine
Division of Rheumatology
Johns Hopkins University School of Medicine
Co-Director
Johns Hopkins Vasculitis Center
Baltimore
MD
利益声明
PS declares that he has no competing interests.
Jaap Van Laar, MD, PhD
Professor of Clinical Rheumatology
Institute of Cellular Medicine
Newcastle University
Newcastle upon Tyne
Honorary Consultant Rheumatologist
The James Cook University Hospital
Middlesbrough
UK
利益声明
JVL is consultant for Roche, the manufacturer of rituximab, and has received speaker fees and reimbursements for attending several conferences.
Robert A. Ortmann, MD
Associate Professor and Director
Division of Rheumatology
University of Arkansas for Medical Sciences
Little Rock
AR
利益声明
RAO declares that he has no competing interests.
鉴别诊断
- 肉芽肿性多血管炎 (GPA)（之前被称为韦格纳肉芽肿）
- 显微镜下多血管炎 (MPA)
- 高嗜酸性粒细胞综合征（HES）
更多 鉴别诊断指南
- 抗中性粒细胞胞浆抗体相关血管炎的管理
- EULAR/ERA-EDTA 关于 ANCA 相关血管炎治疗的建议
更多 指南患者教育信息
何为成人哮喘？
成人哮喘：如何有效治疗？
更多 患者教育信息
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