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Anti-GBM antibody disease: Recurrence after transplantation

Daniel C Brennan, MD, FACP
Andrew Malone, MB, BCh, MRCPI
Section Editor
Barbara Murphy, MB, BAO, BCh, FRCPI
Deputy Editor
Albert Q Lam, MD


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 [1].

There are only six reported cases of symptomatic recurrent anti-GBM disease in the literature [3]. 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 [4].

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 Alport syndrome (hereditary nephritis)", 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) [9]. 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.

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Literature review current through: Nov 2017. | This topic last updated: Jun 13, 2017.
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