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Art of Medicine: Anti-Brush Border Antibody Disease

Anti-Brush Border Antibody Disease
The above painting shows massive tubular basement membrane (electron dense on EM) deposits along proximal tubules with sparing of the distal tubules.  This is a finding that can be seen by electron microscopy in anti-brush border antibody disease (ABBA).   Tubular basement membrane deposits are also seen in lupus nephritis (all classes), IgG4-associated kidney disease, idiopathic hypocomplementemic interstitial nephritis, polyomavirus nephritis (BK virus or JC virus), or in response to certain medications (eculizumab, NSAIDS, and others).   Interstitial inflammation is not prominent in ABBA disease, as it is with other pathologies with tubular basement membrane deposits (such as IgG4-associated disease, drug reactions,...

Twitter Poll (January 16, 2019)

ABBA (Anti-Brush Border Antibody Disease), twitter poll, arkana laboratories, renal pathology
ANSWER: B The target of the circulating ABBA autoantibodies is LDL receptor-related protein 2 (LRP2) also known as “Megalin”. ABBA is a rare autoimmune disease associated with acute kidney injury and tubulointerstitial damage.  Histologically, IgG–positive immune deposits are found in tubular basement membranes by immunofluorescence (IF).  When indirect IF is done using patient’s serum and normal kidney controls, there is reaction of the autoantibodies with the proximal tubular brush borders. REFERENCE: Larsen CP, et al. LDL-Receptor Related Protein 2 (Megalin) as a Target Antigen in Human Kidney Anti-Brush Border Antibody Disease. J Am Soc Nephrol, 2018; 29:644-653    

Anti-Brush Border Antibody Disease

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...

Anti-Brush Border Antibody Disease IF

These photomicrographs show the classic immunofluorescence staining pattern for anti-LRP2 nephropathy, a disease that leads to severe renal tubular injury and is associated with circulating autoantibodies to the tubular brush border protein LRP2/megalin. The images show granular IgG staining along proximal tubular basement membrane, Bowman’s capsule, and segmental glomerular basement membrane staining. The presence of IgG-containing TBM immune deposits, particularly in elderly patients, should alert the clinician and pathologist to the possibility of this disorder. Similar tubular deposits can be seen in the setting of lupus nephritis and IgG4-related disease. Tubular deposits in the setting of lupus generally correlate with...

Diagnose This! (February 13, 2017)

Please pick the best answer. These images show characteristic findings of which of the following diseases: A) IgG4-Related Disease B) Lupus Nephritis C) Sjögren’s Syndrome-Related Tubulointerstitial Injury D) Anti-Brush Border Antibody Tubulointerstitial Nephritis E) Aristolochic Acid Nephropathy F) Mesoamerican Nephropathy Answer: D. Immune complex tubulointerstitial nephritis due to autoantibodies to the proximal tubule brush border is a recently described entity arising from IgG autoantibodies targeting the proximal tubule brush border. Associated immune complex deposits were also reported within the proximal tubule basement membranes and focal, segmental subepithelial space, reminiscent of early membranous glomerulopathy. Further reading: