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Goodpasture 病

最后审阅: 23 Jun 2024
最后更新: 01 Dec 2023

小结

定义

病史和体格检查

关键诊断因素

  • 尿量减少
  • 咯血
  • 水肿
完整详情

其他诊断因素

  • 男性
  • 20至30岁或60至70岁
  • 呼吸短促
  • 咳嗽
  • 发热
  • 恶心
  • 肺部听诊湿啰音
完整详情

危险因素

  • HLA-DRB1或DR4
  • 吸烟
  • 碳氢化合物、有机溶剂、重金属暴露
  • 碎石术治疗史
  • 近期呼吸道感染
  • 免疫治疗
  • Alport 综合征患者肾移植后
完整详情

诊断性检查

首要检查

  • 肾功能检查
  • 肾脏活检
  • 抗肾小球基底膜(抗-GBM)抗体滴度
  • 抗中性粒细胞胞质抗体 (ANCA)
  • 血清补体(C3和C4)
  • 抗核抗体
  • 冷球蛋白
  • 肝炎病毒检测
  • 抗链球菌溶血素O滴度
  • 凝血筛查
完整详情

治疗流程

初步治疗

疑似急进性肾小球肾炎

急症处理

可逆性肾病或任何肺部受累

可逆性肾病且无肺部受累证据

持续性治疗

初始治疗无应答

撰稿人

作者

Tingting Li, MD, MSCI, FASN

Professor of Medicine

Director, Glomerular Diseases Program

Division of Nephrology

Washington University School of Medicine in St Louis

St Louis

MO

利益声明

TL receives research support from Travere Therapeutics, Genentech, Omeros Corporation, and the US NIH/NIAID, and reports consultancy agreements with ChemoCentryx, Travere Therapeutics, Aurinia Pharmaceuticals, Calliditas Therapeutics, and GlaxoSmithKline.

Christina Mariyam Joy, MD

Assistant Professor of Medicine

Division of Nephrology

Froedtert and Medical College of Wisconsin

Milwaukee

WI

利益声明

CMJ declares that she has no competing interests.

鸣谢

Dr Tingting Li and Dr Christina Mariyam Joy would like to gratefully acknowledge Dr Richard G. Phelps, Professor Andre A. Kaplan and Dr Michael S. Gersch, previous contributors to this topic. RGP and MSG declare that they have no competing interests. AAK has received honoraria in 2013 to speak about plasma exchange from Gambro and Alexion (Gambro manufactures products for plasma exchange and Alexion manufactures a complement cascade inhibitor).

同行评议者

Dorin-Bogdan Borza, PhD

Assistant Professor of Medicine

Division of Nephrology

Department of Medicine

Vanderbilt University School of Medicine

Nashville

TN

利益声明

DBB declares that she has no competing interests.

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