Hydroxychloroquine-induced podocytopathy mimicking Fabry disease

BMJ Case Rep. 2019 May 13;12(5):e228876. doi: 10.1136/bcr-2018-228876.


Hydroxychloroquine (HCQ) is largely prescribed as an immunomodulator to prevent systemic diseases flares in patients with systemic lupus erythematous, rheumatoid arthritis, Sjogren's disease. Among reported side effects, HCQ can accumulate in lysosomes and induced phospholipidosis. Here, we report an HCQ-induced podocytopathy mimicking Fabry disease (FD). They share the same histological lesions: cytoplasmic vacuolisation of the podocytes and zebra bodies on light and electronic microscopy. FD has been ruled out by measuring enzymatic activity and genetic test. The persistence of proteinuria after immunological remission of a systemic disease treated with HCQ could suggest this HCQ-induced podocytopathy.

Keywords: pathology; proteinurea; renal system; unwanted effects/adverse reactions.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antirheumatic Agents / adverse effects*
  • Diagnosis, Differential
  • Fabry Disease / diagnosis
  • Female
  • Humans
  • Hydroxychloroquine / adverse effects*
  • Lupus Nephritis / chemically induced*
  • Lupus Nephritis / pathology
  • Male
  • Nephrotic Syndrome / chemically induced*
  • Nephrotic Syndrome / pathology


  • Antirheumatic Agents
  • Hydroxychloroquine