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Anti-GBM antibody disease: Recurrence after transplantation

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

OVERVIEW

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]. However, most patients remain asymptomatic [1].

There are only six reported cases of symptomatic recurrent anti-GBM disease in the literature [2]. A related phenomenon is the development of de novo anti-GBM disease in patients with hereditary nephritis (Alport syndrome), leading to graft loss in up to 5 percent of recipients [3].

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 hereditary nephritis is also discussed elsewhere. (See "Clinical manifestations, diagnosis and treatment of hereditary nephritis (Alport syndrome)", section on 'Anti-GBM antibody disease'.)

PATHOGENESIS

The target glomerular basement antigen for both recurrent anti-glomerular basement membrane (GBM) disease and anti-GBM disease following transplantation in patients with hereditary nephritis is the noncollagenous-1 (NC1) domain of the alpha 3 chain of collagen IV [4-6]. Collagen IV is a family of six alpha chains (alpha-1 through alpha-6) [7]. The alpha-3NC1 antigen is specifically implicated in Goodpasture’s disease. The alpha-3NC1 monomer is assembled into the collagen IV network through the association of the alpha-3, alpha-4, and alpha-5 chains to form a triple helical protomer and through the oligomerization of alpha-345 protomers by means of end-to-end associations and intertwining of triple helixes.

 

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Literature review current through: Nov 2016. | This topic last updated: Thu Jun 04 00:00:00 GMT+00:00 2015.
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References
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