Hydroxychloroquine cardiotoxicity in systemic lupus erythematosus: a report of 2 cases and review of the literature

Semin Arthritis Rheum. 2004 Apr;33(5):336-51. doi: 10.1016/j.semarthrit.2003.09.012.


Background: Hydroxychloroquine (HCQ) is extensively used in the long-term treatment of systemic lupus erythematosus (SLE). Although considered by clinicians to be relatively safe, serious side effects have been documented in the literature. Retinotoxicity has received the most attention, whereas neuromyotoxicity and cardiotoxicity have been described in isolated case reports. We present 2 cases of potential cardiotoxicity occurring in patients with SLE while receiving long-term HCQ therapy.

Objective: To review the incidence, presentation, and mechanism of serious antimalarial toxicity, and to discuss the impact of HCQ on cardiac health in SLE.

Methods: The authors reviewed the English-language literature from 1948 to December 2002 using Medline databases.

Results: In addition to our patients, there are 2 published cases of biopsy-proven HCQ cardiotoxicity in the English-language literature. Both occurred in patients with SLE. The literature indicates that antimalarial cardiotoxicity may be of particular importance in patients with SLE given their already increased cardiac risk due to primary heart disease and accelerated atherosclerosis. Endomyocardial biopsy reveals a constellation of findings including vacuolar myopathy, myeloid bodies, and curvilinear bodies.

Conclusions: As HCQ use among SLE patients increases, clinicians should be alert to the possibility of antimalarial cardiotoxicity. The potential severity and reversibility of this complication underscore the importance of timely diagnosis. The cases presented here, one with biopsy and one without, illustrate the utility of endomyocardial biopsy in HCQ-treated SLE patients with cardiac complaints to ensure accurate diagnosis and appropriate management.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antirheumatic Agents / adverse effects*
  • Antirheumatic Agents / therapeutic use
  • Cardiomyopathies / chemically induced*
  • Female
  • Heart / drug effects
  • Heart Conduction System / drug effects
  • Humans
  • Hydroxychloroquine / adverse effects*
  • Hydroxychloroquine / therapeutic use
  • Lupus Erythematosus, Systemic / drug therapy*
  • Middle Aged
  • Myocardium / pathology
  • Risk Factors
  • Time Factors


  • Antirheumatic Agents
  • Hydroxychloroquine