A Growing Concern: Cardiomyopathy with Long Term Hydroxychloroquine Use
Bhavna Abbi2, Sneha N. Patel1, Irene Blanco1, Anand Kumthekar1, Daniel Schwartz3. 1Rheumatology, Montefiore Medical Center, Moses Division, Bronx, New York, United States, 2Internal Medicine, St. John's Riverside Hospital, Yonkers, New York, United States, 3Pathology, Montefiore Medical Center, Moses Division, Bronx, New York, United States
Purpose of Study Hydroxychloroquine (HCQ) is used widely in the treatment of Systemic Lupus Erythematosus (SLE). Cardiac toxicity from HCQ is exceedingly rare and may lead to myocardial hypertrophy, cardiomyopathy or conduction abnormalities. We present a case of a 42 year-old female with SLE treated with long-standing HCQ who was found to have acute onset heart failure attributed to HCQ toxicity.
Methods Used A 42 year-old female with history of hypertension, chronic kidney disease and SLE presented with a six month history of shortness of breath. She was diagnosed with SLE in 2008 and has been on 400mg of HCQ (5.4 mg/kg) without periodic monitoring. On presentation, her blood pressure was 210/135 mmHg and exam was significant for hyperpigmented lesions on the face, hands and hard palate. Electrocardiogram (EKG) showed nonspecific t-wave inversions and QT prolongation. Transthoracic Echocardiogram (TTE) revealed severe bi-atrial dilation and moderate bi-ventricular hypokinesis. Her TTE findings were atypical for hypertensive cardiomyopathy and thus, a myocardial biopsy was performed. Endomyocardial biopsy showed electron microscopic evidence of HCQ toxicity (Figure 1). Hydroxychloroquine was thus discontinued indefinitely.
Summary of Results Hydroxychloroquine-induced cardiotoxicity is a rare condition seen with long term HCQ use. On review of the literature, the number of cases prior to July 2017 ranges between 40 and 70, and only half of these are biopsy proven. While our patient developed cardiac toxicity after ten years of intermittent HCQ use, varying dosages and durations can lead to cardiac toxicity. Hartmann et al reported toxic effects from as little as 290g of cumulative dose to as much as 4380g of HCQ. Similarly, cardiac damage can develop after a mean of 10 years in some patients, while in others, 30 years of therapy was noted before the development of heart failure. To prevent cardiac manifestations, sources have recommended screening patients on HCQ using routine EKG and TTE. However, no consensus has been established regarding screening modalities and larger studies are needed to elucidate the utility of this practice.
Conclusions We report a case of biopsy-proven HCQ cardiac toxicity.
Figure 1: Electron micrograph of myocardium showing myelin bodies (round, concentric laminations within the sarcomeres), typical of HCQ toxicity.
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