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Anti-glomerular basement membrane (Goodpasture's) disease

最后审阅: 15 Nov 2025
最后更新: 20 Nov 2024

小结

定义

病史和体格检查

关键诊断因素

  • reduced urine output
  • haemoptysis
  • oedema
完整详情

其他诊断因素

  • male sex
  • age 20 to 30 years or 60 to 70 years
  • shortness of breath
  • cough
  • fever
  • nausea
  • crackles on lung examination
完整详情

危险因素

  • HLA-DRB1 or DR4
  • smoking
  • exposure to hydrocarbons, organic solvents, heavy metals
  • history of lithotripsy
  • recent respiratory infections
  • immunotherapy
  • post-renal transplant in patients with Alport syndrome
完整详情

诊断性检查

首要检查

  • renal function testing
  • renal biopsy
  • anti-glomerular basement membrane (anti-GBM) antibody titre
  • anti-neutrophil cytoplasmic antibodies (ANCA)
  • serum complement (C3 and C4)
  • antinuclear antibody
  • cryoglobulins
  • hepatitis panel
  • anti-streptolysin O titre
  • clotting screen
完整详情

治疗流程

初步治疗

suspected rapidly progressive glomerulonephritis

急症处理

reversible renal disease or any pulmonary involvement

irreversible renal disease and no evidence of pulmonary involvement

持续性治疗

unresponsive to initial treatment

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, Omeros Corporation, and the US NIH/NIAID, and reports consultancy agreements with ChemoCentryx, Travere Therapeutics, Amgen, Aurinia Pharmaceuticals, Calliditas Therapeutics, and GlaxoSmithKline. She also serves as a board member of GlomCon and a member of the ASN Publications Committee.

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.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

参考文献

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

关键文献

Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Jul 7;64(7):1535-50. 摘要

Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. 2002 Dec;95(12):1411-8. 摘要

Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999 Nov;10(11):2446-53.全文  摘要

Connelly-Smith L, Alquist CR, Aqui NA, et al. Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the Writing Committee of the American Society for Apheresis: the ninth special issue. J Clin Apher. 2023 Apr;38(2):77-278. 摘要

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-S276.全文  摘要

参考文献

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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  • Diretrizes

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