小结
定义
病史和体格检查
关键诊断因素
- reduced urine output
- haemoptysis
- oedema
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
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
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
治疗流程
suspected rapidly progressive glomerulonephritis
reversible renal disease or any pulmonary involvement
irreversible renal disease and no evidence of pulmonary involvement
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
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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更多 鉴别诊断Diretrizes
- Guidelines on the use of therapeutic apheresis in clinical practice - ninth special issue
- Clinical practice guideline for glomerulonephritis
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