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ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review

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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.

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Data availability

The clinical data (with the patient’s identifiable information deleted), and original pathology images of conventional light microscopy, immunohistochemical stains, immunofluorescence, special stains, electron microscopy, and mass spectrometry report can be accessed upon request.

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Acknowledgements

The authors would like to thank Fredrik Hans Skarstedt for his great assistance in preparing the figures.

Funding

The authors received no financial support for the work on this article.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA

    Muhammad Shaheen & Shunhua Guo

  2. Division of Critical Care Medicine, Advocate Aurora Health, St. Luke’s Medical Center, Milwaukee, WI, USA

    Anchit Bharat

  3. Department of Medicine, Division of Nephrology, Indiana University School of Medicine, Indianapolis, IN, USA

    Allon N. Friedman

Authors
  1. Muhammad Shaheen
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  2. Anchit Bharat
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  3. Allon N. Friedman
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  4. Shunhua Guo
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Correspondence to Shunhua Guo.

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Shaheen, M., Bharat, A., Friedman, A.N. et al. ALECT2 amyloidosis with concurrent IgG4-related interstitial nephritis, membranous nephropathy and diabetic kidney disease: a case report and literature review. J Nephrol (2024). https://doi.org/10.1007/s40620-024-01924-9

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  • DOI: https://doi.org/10.1007/s40620-024-01924-9

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