Cobarro Gálvez Lucía, Valbuena-López Silvia, Prieto Moriche Elsa, Ruiz Bravo-Burguillos Elena, Pérez David Esther
Cardiology Department, La Paz University Hospital, Paseo de la Castellana, 261, 28046 Madrid, Spain.
Anatomical Pathology Department, La Paz University Hospital, Paseo de la Castellana, 261, 28046 Madrid, Spain.
Eur Heart J Case Rep. 2024 Aug 30;8(8):ytae404. doi: 10.1093/ehjcr/ytae404. eCollection 2024 Aug.
Hydroxychloroquine (HCQ) is a disease-modifying antirheumatic used in rheumatological diseases such as systemic lupus erythematosus. Long-term exposure to HCQ results in drug accumulation and predisposes to adverse effects.
We present the case of a 45-year-old woman with long-term treatment with HCQ who presented to the Emergency Department with acute heart failure. Transthoracic echocardiogram, previously normal, showed severe biventricular hypertrophy and biventricular systolic dysfunction. Cardiac magnetic resonance (CMR) confirmed the previous findings and showed elevated native T1 and T2 values, elevated extracellular volume, and extensive mid-wall late gadolinium enhancement (LGE). Infiltrative cardiomyopathy was suspected, and endomyocardial biopsy performed. Light microscopy showed myocyte hypertrophy and vacuolar change and absence of lymphocytic inflammatory infiltrates. The diagnosis of HCQ-induced cardiomyopathy was established, and the drug was withdrawn. A CMR performed 1 year later showed normal systolic function of both ventricles and normalization of T2 values, reflecting resolution of myocardial oedema. However, severe hypertrophy, elevated native T1 values, and LGE persisted.
Our case shows that although discontinuation of the drug stops the progression of the disease, established myocardial structural damage persists. Early diagnosis of this entity is therefore essential to improve prognosis.
羟氯喹(HCQ)是一种用于治疗系统性红斑狼疮等风湿性疾病的改善病情抗风湿药。长期使用HCQ会导致药物蓄积并易引发不良反应。
我们报告一例45岁长期接受HCQ治疗的女性患者,她因急性心力衰竭就诊于急诊科。之前正常的经胸超声心动图显示严重的双心室肥厚和双心室收缩功能障碍。心脏磁共振成像(CMR)证实了之前的检查结果,并显示固有T1和T2值升高、细胞外容积增加以及广泛的中层心肌延迟钆增强(LGE)。怀疑为浸润性心肌病,遂进行心内膜心肌活检。光学显微镜检查显示心肌细胞肥大和空泡样改变,无淋巴细胞炎性浸润。确诊为HCQ诱发的心肌病,并停用该药。1年后进行的CMR显示双心室收缩功能正常,T2值恢复正常,提示心肌水肿消退。然而,严重肥厚、固有T1值升高和LGE仍然存在。
我们的病例表明,尽管停药可阻止疾病进展,但已形成的心肌结构损伤仍然存在。因此,早期诊断该疾病对于改善预后至关重要。