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December 21, 2017

Anti-Brush Border Antibody Disease

Anti-LPR2 Nephropathy

Anti-LRP2 nephropathy is a form of kidney disease primarily affecting the elderly in which progressive renal tubular injury is seen in association with circulating autoantibodies to the tubular brush border protein LRP2 (aka megalin). Histopathologically, it is characterized by IgG immune complexes in the tubular basement membrane. Similar tubular basement membrane deposits can be seen in other diseases such as lupus nephritis and IgG4-related disease. However, TBM staining for LRP2 is unique to cases with anti-LPR2 nephropathy. Normally, LRP2 is restricted to the apical membrane of the proximal tubules. LRP2 staining along the tubular basement membranes is abnormal indicates the presence of anti-LRP2 nephropathy.

Reference: J Am Soc Nephrol. 2017 Oct 26. [Epub ahead of print] PMID: 29074737

Anti-LPR2 Nephropathy, Anti-Brush Border Antibody Disease