Anti-glomerular basement membrane (Goodpasture's) disease

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Última revisión: 12 Dec 2025

Última actualización: 20 Nov 2024

Goodpasture's disease is defined by the presence of autoantibodies to the alpha-3 chain of type IV collagen. It presents with rapidly progressive renal dysfunction often combined with pulmonary haemorrhage.

Simultaneous haemoptysis and renal failure (the pulmonary-renal syndrome) has many causes, but where the renal disease is a rapidly progressive glomerulonephritis, about 15% of cases will be Goodpasture's disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis.

Rapid diagnosis by early renal biopsy and serological testing is essential, because appropriate treatment given before renal damage is advanced can result in an excellent prognosis.

Treatment is by removal of circulating antibodies using plasma exchange combined with agents to prevent their continued production.

Definición

Goodpasture’s disease, also known as anti-glomerular basement membrane antibody (anti-GBM) disease, is an important cause of pulmonary-renal syndrome (Goodpasture’s syndrome).[1] Pulmonary-renal syndrome consists of glomerulonephritis and pulmonary haemorrhage. Goodpasture’s disease is caused by an autoantibody to the alpha-3 chain of type IV collagen. This type of collagen is principally found in the basement membranes of alveoli and glomeruli.

Anamnesis y examen

Principales factores de diagnóstico

reduced urine output
haemoptysis
oedema

Todos los datos

Otros factores de diagnóstico

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

Todos los datos

Factores de riesgo

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

Todos los datos

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

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

Todos los datos

Algoritmo de tratamiento

Inicial

suspected rapidly progressive glomerulonephritis

Agudo

reversible renal disease or any pulmonary involvement

irreversible renal disease and no evidence of pulmonary involvement

En curso

unresponsive to initial treatment

Colaboradores

Autores

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

Divulgaciones

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

Divulgaciones

CMJ declares that she has no competing interests.

Agradecimientos

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).

Revisores por pares

Dorin-Bogdan Borza, PhD

Assistant Professor of Medicine

Division of Nephrology

Department of Medicine

Vanderbilt University School of Medicine

Nashville

Раскрытие информации

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.

Список литературы

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.

Основные статьи

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

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

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

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. Аннотация

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.Полный текст Аннотация

Статьи, указанные как источники

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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Рекомендации
- Guidelines on the use of therapeutic apheresis in clinical practice - ninth special issue
- Clinical practice guideline for glomerulonephritis
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Anti-glomerular basement membrane (Goodpasture's) disease

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

Otros factores de diagnóstico

Factores de riesgo

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

Algoritmo de tratamiento

suspected rapidly progressive glomerulonephritis

reversible renal disease or any pulmonary involvement

irreversible renal disease and no evidence of pulmonary involvement

unresponsive to initial treatment

Colaboradores

Autores

Tingting Li, MD, MSCI, FASN

Divulgaciones

Christina Mariyam Joy, MD

Divulgaciones

Agradecimientos

Revisores por pares

Dorin-Bogdan Borza, PhD

Раскрытие информации

Peer reviewer acknowledgements

Disclosures

Список литературы

Основные статьи

Статьи, указанные как источники

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

Отличия

Рекомендации

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись или оформите подписку, чтобы получить полноценный доступ к BMJ Best Practice

Войдите в учетную запись, чтобы получить полноценный доступ к BMJ Best Practice