Anti-GBM antibody disease: Recurrence after transplantation
- Daniel C Brennan, MD, FACP
Daniel C Brennan, MD, FACP
- Editor-in-Chief — Nephrology
- Section Editor — Renal Transplantation
- Professor of Medicine
- Medical Director and Co-Director of the Comprehensive Transplant Center, Department of Internal Medicine, Division of Nephrology
- Johns Hopkins Medical School
- Andrew Malone, MB, BCh, MRCPI
Andrew Malone, MB, BCh, MRCPI
- Assistant Professor of Medicine, Division of Nephrology
- Department of Medicine, Washington University School of Medicine
Anti-glomerular basement membrane (GBM) antibody disease may progress to end-stage renal failure, requiring either dialysis or renal transplantation. The incidence of recurrent linear immunoglobulin G (IgG) staining in the transplant may be as high as 50 percent [1,2]. However, most patients remain asymptomatic .
There are only six reported cases of symptomatic recurrent anti-GBM disease in the literature . A related phenomenon is the development of de novo anti-GBM disease in patients with Alport syndrome (also known as Alport posttransplant anti-GBM nephritis), which can lead to graft loss in up to 5 percent of recipients .
This topic reviews recurrent anti-GBM disease after transplantation. The pathogenesis, diagnosis, and treatment of anti-GBM disease are discussed elsewhere. (See "Pathogenesis and diagnosis of anti-GBM antibody (Goodpasture's) disease" and "Treatment of anti-GBM antibody (Goodpasture's) disease".)
De novo anti-GBM disease following transplantation in patients with Alport syndrome is also discussed elsewhere. (See "Clinical manifestations, diagnosis, and treatment of hereditary nephritis (Alport syndrome)", section on 'Anti-GBM antibody disease'.)
The target glomerular basement membrane (GBM) antigens for recurrent anti-GBM disease are the noncollagenous-1 (NC1) domains of the alpha-3 and alpha-5 chains of collagen IV (alpha3[IV]NC1 and alpha5[IV]NC1) [5-8]. Collagen IV is a family of six alpha chains (alpha-1 through alpha-6) . The alpha-3, alpha-4, and alpha-5 chains form a triple helical protomer, and oligomerization of alpha-345 protomers, by means of NC1-NC1 end-to-end associations, forms the hexameric NC1 domain.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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