Hydroxychloroquine Cardiotoxicity Mimicking Transthyretin Cardiac Amyloidosis in a Patient With HFpEF
6 affiliations
- 1Department of Internal Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
- 2Divison of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA.
- 3Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
- 4Department of Diagnostic Radiology, Medical University of South Carolina, Charleston, South Carolina, USA.
- 5Divison of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA; Division of Cardiology, Ralph H. Johnson Veterans Affairs Health System, Charleston, South Carolina, USA.
- 6Divison of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA. Electronic address: [email protected].
Abstract
Background: Hydroxychloroquine (HCQ) is an immunomodulatory drug used in systemic lupus erythematosus and other rheumatologic disorders, with rare but potentially severe cardiotoxic effects.
Case Summary: A 77-year-old woman with a history of systemic lupus erythematosus and heart failure with preserved ejection fraction presented with subacute worsening chest pain, dyspnea, palpitations, and neuropathy. Electrocardiogram showed low voltage; echocardiogram revealed moderate concentric left ventricular hypertrophy with reduced global longitudinal strain without apical sparing. Coronary angiography was normal. Technetium-99m pyrophosphate scintigraphy showed focal grade 2 uptake. Endomyocardial biopsy revealed widespread myocyte hypertrophy and lysosomal curvilinear bodies consistent with HCQ cardiotoxicity.
Discussion: HCQ is a long-term medication used in connective tissue diseases. Patients with rheumatologic conditions are at increased risk for heart failure with preserved ejection fraction independent of HCQ use. Recognition of insidious organ toxicity can be challenging. HCQ toxicity can cause infiltrative pathology and mimic transthyretin cardiac amyloidosis. Endomyocardial biopsy is key for definitive diagnosis.
Take-Home Message: In patients with an equivocally positive technetium-99m pyrophosphate scan and chronic HCQ use, endomyocardial biopsy should be considered to differentiate drug toxicity from transthyretin cardiac amyloidosis.
Related
- Effect of Transthyretin Stabilizer in Transthyretin Cardiac Amyloidosis
- Gender and Racial Distribution of Transthyretin Stabilizer Therapy in Patients With Transthyretin Cardiac Amyloidosis.
- A Progressive 99mTc-MDP Uptake in Transthyretin Cardiac Amyloidosis
- The Progressive Cardiac Uptake of 99mTc-MDP as a Clue to Transthyretin Amyloidosis
- Transcatheter Mitral Edge-to-edge Repair in Patients with Transthyretin Cardiac Amyloidosis: A Four-case Series
BibTeX
@article{asempah2026hydroxychloroquine,
title = {{Hydroxychloroquine Cardiotoxicity Mimicking Transthyretin Cardiac Amyloidosis in a Patient With HFpEF}},
author = {Moses Asempah and Elie Kozaily and Izabela Abissi and Clarence Silvis and Corynne Caballero and Deven Champaneri and Sheldon E Litwin and Matthew J Belanger},
journal = {JACC Case Reports},
year = {2026},
month = oct,
doi = {10.1016/j.jaccas.2026.110638},
url = {https://doi.org/10.1016/j.jaccas.2026.110638},
note = {CC BY-NC-ND 4.0},
}
RIS
TY - JOUR
TI - Hydroxychloroquine Cardiotoxicity Mimicking Transthyretin Cardiac Amyloidosis in a Patient With HFpEF
AU - Moses Asempah
AU - Elie Kozaily
AU - Izabela Abissi
AU - Clarence Silvis
AU - Corynne Caballero
AU - Deven Champaneri
AU - Sheldon E Litwin
AU - Matthew J Belanger
PY - 2026
DA - 2026/10/01
JO - JACC Case Reports
DO - 10.1016/j.jaccas.2026.110638
UR - https://doi.org/10.1016/j.jaccas.2026.110638
AB - Background: Hydroxychloroquine (HCQ) is an immunomodulatory drug used in systemic lupus erythematosus and other rheumatologic disorders, with rare but potentially severe cardiotoxic effects. Case Summary: A 77-year-old woman with a history of systemic lupus erythematosus and heart failure with preserved ejection fraction presented with subacute worsening chest pain, dyspnea, palpitations, and neuropathy. Electrocardiogram showed low voltage; echocardiogram revealed moderate concentric left ventricular hypertrophy with reduced global longitudinal strain without apical sparing. Coronary angiography was normal. Technetium-99m pyrophosphate scintigraphy showed focal grade 2 uptake. Endomyocardial biopsy revealed widespread myocyte hypertrophy and lysosomal curvilinear bodies consistent with HCQ cardiotoxicity. Discussion: HCQ is a long-term medication used in connective tissue diseases. Patients with rheumatologic conditions are at increased risk for heart failure with preserved ejection fraction independent of HCQ use. Recognition of insidious organ toxicity can be challenging. HCQ toxicity can cause infiltrative pathology and mimic transthyretin cardiac amyloidosis. Endomyocardial biopsy is key for definitive diagnosis. Take-Home Message: In patients with an equivocally positive technetium-99m pyrophosphate scan and chronic HCQ use, endomyocardial biopsy should be considered to differentiate drug toxicity from transthyretin cardiac amyloidosis.
SN - 2666-0849
ER -