Goodpasture disease - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Goodpasture 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 hemorrhage.
Simultaneous hemoptysis 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 disease, with the remainder mostly antineutrophil cytoplasmic antibody vasculitis.
Rapid diagnosis by early renal biopsy and serologic 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.

Immunofluorescence of renal biopsy staining for IgG in a linear pattern in patient with antiglomerular basement membrane (anti-GBM) disease

From the collection of Michael S. Gersch

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Images

Immunofluorescence of renal biopsy staining for IgG in a linear pattern in patient with antiglomerular basement membrane (anti-GBM) disease

Citations

Key Articles

Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003 Jul 7;64(7):1535-50.[Abstract]
Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture's syndrome. South Med J. 2002 Dec;95(12):1411-8.[Abstract]
Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999 Nov;10(11):2446-53.[Abstract][Full Text]
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.[Abstract]
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.[Abstract][Full Text]

Referenced Articles

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20. Prendecki M, Clarke C, Cairns T, et al. Anti-glomerular basement membrane disease during the COVID-19 pandemic. Kidney Int. 2020 Sep;98(3):780-81.[Full Text]
21. Phelps R, Winston JA, Wynn D, et al. Incidence, management, and outcomes of autoimmune nephropathies following alemtuzumab treatment in patients with multiple sclerosis. Mult Scler. 2019 Aug;25(9):1273-88.[Abstract][Full Text]
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24. Al-Chalabi S, Wu HHL, Chinnadurai R, et al. Etanercept-induced anti-glomerular basement membrane disease. Case Rep Nephrol Dial. 2021 Sep-Dec;11(3):292-300.[Abstract][Full Text]
25. Browne G, Brown PA, Tomson CR, et al. Retransplantation in alport post-transplant anti-GBM disease. Kidney Int. 2004 Feb;65(2):675-81.[Abstract][Full Text]
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27. 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.[Abstract]
28. 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.[Abstract][Full Text]
29. Swaak AJ, Groenwold J, Bronsveld W. Predictive value of complement profiles and anti-dsDNA in systemic lupus erythematosus. Ann Rheum Dis. 1986 May;45(5):359-66.[Abstract]
30. Reggiani F, L'Imperio V, Calatroni M, et al. Goodpasture syndrome and anti-glomerular basement membrane disease. Clin Exp Rheumatol. 2023 Apr;41(4):964-74.[Abstract][Full Text]
31. van Daalen EE, Jennette JC, McAdoo SP, et al. Predicting outcome in patients with anti-GBM glomerulonephritis. Clin J Am Soc Nephrol. 2018 Jan 6;13(1):63-72.[Abstract][Full Text]
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33. Levy JB, Turner AN, Rees AJ, et al. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001 Jun 5;134(11):1033-42.[Abstract]
34. Kant S, Kronbichler A, Sharma P, et al. Advances in understanding of pathogenesis and treatment of immune-mediated kidney disease: a review. Am J Kidney Dis. 2022 Apr;79(4):582-600.[Abstract][Full Text]
35. Hu W, Liu Z, Ji D, et al. Staphylococcal protein A immunoadsorption for Goodpasture's syndrome in four Chinese patients. J Nephrol. 2006 May-Jun;19(3):312-7.[Abstract]
36. Bygren P, Freiburghaus C, Lindholm T, et al. Goodpasture's syndrome treated with staphylococcal protein A immunoadsorption. Lancet. 1985 Dec 7;2(8467):1295-6.[Abstract]
37. Huart A, Josse AG, Chauveau D, et al. Outcomes of patients with Goodpasture syndrome: a nationwide cohort-based study from the French Society of Hemapheresis. J Autoimmun. 2016 Sep;73:24-9.[Abstract]