IMAGES

A Case of Cardiomyopathy With Long-term

Hydroxychloroquine Use
Bhavna Abbi, MD,* Sneha Patel, MD,† Anand Kumthekar, MD,†
Daniel Schwartz, MD,‡ and Irene Blanco, MD†

A 42-year-old woman with medical history of uncontrolled hy-

pertension, stage 5 chronic kidney disease with no prior renal
biopsy, and systemic lupus erythematosus (SLE) presented to our
institution with a 6-month history of shortness of breath and ana-
sarca. She had not received routine rheumatologic care since her
diagnosis of SLE. In the interim, she received 400 mg daily dosing
of hydroxychloroquine from emergency department encounters.
At the time of presentation, her vitals were significant for a blood
pressure of 210/135 mm Hg and physical examination findings re-
vealing hyperpigmented lesions on the face, palms, dorsum of the
hands, and hard palate. Laboratory studies revealed a creatinine of
2.68 mg/dL (baseline unknown), brain natriuretic peptide of
greater than 15,000 pg/mL, and a troponin T of 0.04 ng/mL. Chest
radiography showed vascular congestion and bilateral pleural ef-
fusions. At this time, the patient's renal function continued to
worsen. Transthoracic echocardiogram revealed severe biatrial
dilation, moderate biventricular hypokinesis, and moderately in-
creased left atrial (15–22 mm Hg) and right atrial (11–20 mm Hg)
pressures. Furosemide drip was started for acute decompensated
heart failure. Left-sided heart catheterization performed revealed
nonobstructive coronaries. After right-sided heart catheterization,
the patient underwent septal biopsy to evaluate the etiology of her
heart failure. Endomyocardial biopsy revealed electron micro-
scopic evidence of hydroxychloroquine toxicity (Fig.). Hydroxy- FIGURE. Endomyocardial biopsy revealing myelin bodies (round,
chloroquine was thus discontinued indefinitely. concentric laminations within the sarcomeres), consistent with
Hydroxychloroquine is a disease-modifying antirheumatic hydroxychloroquine toxicity.
drug and forms the backbone of SLE treatment. Although it has
been considered safe to use, prolonged use has been associated effects from as little as 290 g of cumulative dose to as much as
with toxic side effects such as retinopathy, vacuolar myopathy, 4380 g of hydroxychloroquine. Similarly, cardiac damage can de-
cardiotoxicity, and neuropathy. velop after a mean of 10 years in some patients,4 whereas in
Hydroxychloroquine-induced cardiotoxicity is a rare clinical others, 30 years of therapy was noted before the development of
entity that can be fatal. On review of the literature, the number of heart failure.5 In order to prevent cardiac manifestations, various
cases prior to July 2017 ranges between 40 and 70, and only half sources have recommended screening for patients on hydroxy-
of these cases are biopsy proven.1 Cardiac toxicity is thought to chloroquine using routine electrocardiogram and transthoracic
arise due to myocyte membrane phospholipid disruption and ac- echocardiogram. Unfortunately, no consensus has been established
cumulation within lysosomes causing myocyte necrosis and vacu- regarding screening modalities, and larger studies are needed to
olar myopathy. These changes can cause systolic dysfunction and further elucidate the usefullness of this practice.
conduction abnormalities. If there is a high index of suspicion for
cardiac involvement, biopsy should be performed. Diagnosis is REFERENCES
confirmed by electron microscopy evidence of curvilinear bodies
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cardiotoxicity in a 39-year-old woman with systemic lupus erythematosus
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From the *Department of Internal Medicine, St John's Riverside Hospital, failure in a patient with hydroxychloroquine-induced cardiomyopathy.
Yonkers, NY; †Department of Rheumatology; and ‡Pathology, Montefiore Neth Heart J. 2011;19:482–485.
Medical Center, Bronx, NY.
The authors declare no conflict of interest. 4. Costedoat-Chalumeau N, Hulot JS, Amoura Z, et al. Cardiomyopathy
Correspondence: Bhavna Abbi, MD, Department of Internal Medicine, related to antimalarial therapy with illustrative case report. Cardiology. 2007;
St John's Riverside Hospital, 967 North Broadway, Yonkers, NY 10701. 107:73–80.
E‐mail: bhavna.abbi.1@gmail.com.
Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved. 5. Cotroneo J, Sleik KM, Rene Rodriguez E, et al.
ISSN: 1076-1608 Hydroxychloroquine-induced restrictive cardiomyopathy. Eur J
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