Kidney Podocyte Zebra Bodies after Lung Transplantation for Lymphangioleiomyomatosis

Intern Med. 2023 Jul 1;62(13):1965-1970. doi: 10.2169/internalmedicine.0882-22. Epub 2022 Nov 9.

Abstract

A 55-year-old woman showed progressive renal dysfunction after unilateral deceased-donor lung transplantation for lymphangioleiomyomatosis. A kidney biopsy showed a striped pattern of interstitial fibrosis, suggesting calcineurin inhibitor toxicity, and zebra body accumulation predominantly in the podocytes, characteristics of Fabry disease. Nevertheless, she had no extra-renal symptoms of the disease, and gene testing identified no known pathogenic variant or exon deletion. Our case report and literature review suggest that this atypical lysosomal inclusion may be phospholipidosis induced by sertraline. Potential underlying etiologies linking zebra body deposits may be not only hereditary but also drug-induced phospholipidosis.

Keywords: calcineurin inhibitor; chronic kidney disease; drug-induced phospholipidosis; fabry disease; lung transplantation; zebra body.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Fabry Disease* / genetics
  • Female
  • Humans
  • Kidney / pathology
  • Lung Transplantation* / adverse effects
  • Lymphangioleiomyomatosis* / surgery
  • Podocytes* / pathology