Journal article
ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review
Journal of nephrology, Vol.37(8), pp.2363-2369
11/01/2024
DOI: 10.1007/s40620-024-01924-9
PMID: 38630340
Abstract
Leukocyte chemotactic factor-2 amyloidosis (ALECT2) is a recently described subtype of amyloidosis. IgG4-related disease is a rare fibroinflammatory condition characterized by dense interstitial lymphoplasmacytic infiltrates and fibrosis. Membranous nephropathy and diabetic nephropathy are common causes of nephrotic syndrome. Here we report a 49-year-old Hispanic male patient with diabetes mellitus who presented with jaundice and pruritus. IgG4-related autoimmune pancreatitis was diagnosed through laboratory workup and ampulla biopsy. He subsequently presented with marked lower extremity edema and nephrotic syndrome. Kidney biopsy showed severe interstitial IgG4-positive plasma cell-rich inflammatory infiltrates and interstitial storiform fibrosis. Immunofluorescence microscopy revealed diffuse and finely granular glomerular capillary wall staining for IgG and the glomeruli were negative for anti-phospholipase A2 receptor. Congo red stain was positive for birefringent deposits in the interstitium, arteriolar walls, and glomeruli. Electron microscopy demonstrated subepithelial immune complex-type electron-dense deposits, thickening of glomerular basement membranes (GBM), and randomly oriented fibrils in the mesangium, GBM, and interstitium. Mass spectrometry identified a peptide profile consistent with ALECT2 amyloidosis. This is the first report of a case with concurrence of ALECT2 amyloidosis, IgG4-related disease involving the kidney, membranous nephropathy, and early diabetic kidney injury.
Details
- Title: Subtitle
- ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review
- Creators
- Muhammad Shaheen - Indiana University School of MedicineAnchit Bharat - Aurora St. Luke's Medical CenterAllon N Friedman - Indiana University School of MedicineShunhua Guo - Indiana University School of Medicine
- Resource Type
- Journal article
- Publication Details
- Journal of nephrology, Vol.37(8), pp.2363-2369
- DOI
- 10.1007/s40620-024-01924-9
- PMID
- 38630340
- NLM abbreviation
- J Nephrol
- ISSN
- 1724-6059
- eISSN
- 1724-6059
- Language
- English
- Date published
- 11/01/2024
- Academic Unit
- Pathology
- Record Identifier
- 9985217818302771
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