Chloroquine-induced phospholipidosis of the kidney mimicking Fabry's disease: case report and review of the literature

Hum Pathol. 2003 Mar;34(3):285-9. doi: 10.1053/hupa.2003.36.


A 46-year-old female patient with Sjögren's syndrome, hypertension, and stable chronic renal insufficiency (creatinine [CR], 1.9 to 2.1 mg/dL) had a progressive worsening of renal function (CR, 5.0 mg/dL) after 11 months of chloroquine therapy (155 mg/day; cumulative dose of approximately 51 g). Light microscopy revealed nonspecific angionephrosclerosis. Electron microscopy showed accumulations of lamellated myelinoid material and occasionally also of curvilinear bodies, especially in the glomerular podocytes and to a lesser extent in vascular myothelial and endothelial cells. In the tubular system, mainly protein droplets were stored. Activity of alpha-galactosidase A was normal in isolated leukocytes (56 nmol/mg; range, 33.2 to 109 nmol/mg), ruling out Fabry's disease. Clinical, morphological, and biochemical findings were consistent with chloroquine-associated deterioration of renal function that improved considerably after discontinuation of chloroquine treatment. Adverse effects of chloroquine may aggravate preexisting renal disease. Electron microscopy is a worthwhile tool for establishing the correct diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Chloroquine / adverse effects*
  • Diagnosis, Differential
  • Fabry Disease*
  • Female
  • Humans
  • Hypertension / complications
  • Kidney / ultrastructure
  • Kidney Diseases / chemically induced*
  • Kidney Diseases / pathology
  • Kidney Failure, Chronic / complications
  • Kidney Glomerulus / ultrastructure
  • Leukocytes / enzymology
  • Microscopy, Electron
  • Middle Aged
  • Nephrosclerosis
  • Phospholipids / metabolism*
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / drug therapy*
  • alpha-Galactosidase / metabolism


  • Phospholipids
  • Chloroquine
  • alpha-Galactosidase