Clinicopathologic Features of Antibrush Border Antibody Disease

All About ABBA: New Insights Into Antibrush Border Antibody Disease

“…A characteristic finding was the presence of segmental membranous features with glomerular subepithelial deposits detected in nearly 80% of biopsies, as well as deposits in Bowman’s capsule in 72%. 7 This is surprising, given that LRP2 expression has not been definitively demonstrated by highly sensitive molecular techniques in normal human podocytes or flat parietal epithelial cells; moreover, staining for LRP2 was negative in the distribution of the glomerular deposits in all but one case. These results suggest potential plasticity in gene expression among a subset of human parietal epithelial cells and podocytes.…”

“…The authors now enlarge their series to 67 patients gleaned from the Arkana Laboratory archives, corresponding to a biopsy incidence of only 0.05%. 7 This large cohort provides valuable new insights into the clinicopathologic characteristics and outcomes of this rare, underrecognized disease. 7 …”

“… 7 This large cohort provides valuable new insights into the clinicopathologic characteristics and outcomes of this rare, underrecognized disease. 7 …”

“…Presentations were most often acute kidney injury and/or progressive chronic kidney disease, with mean estimated glomerular filtration rate of 26.6 ml/min and median serum creatinine of 2.7 mg/dl. 7 Surprisingly, despite the targeted injury to proximal tubules, no patient manifested overt Fanconi syndrome. Although 95% of patients had proteinuria >500 mg, only 22% had nephrotic syndrome that was related to associated glomerular diseases.…”

“…A major new observation from this cohort was the existence of concurrent pathologic diagnoses in 57% of biopsies. 7 Coexistent glomerular diseases were diverse, including global membranous nephropathy, minimal change disease, primary focal segmental glomerulosclerosis, diabetic glomerulosclerosis, IgA nephropathy, proliferative lupus nephritis, crescentic pauci-immune glomerulonephritis and light chain amyloidosis. Tubulointerstitial diseases included acute or granulomatous interstitial nephritis and rare interstitial infiltration by B-cell lymphoma.…”