Durrance Richard Jesse, Movahedian Malahat, Haile Worku, Teller Katerina, Pinsker Richard
Department of Medicine, Jamaica Hospital Medical Center, 8900 Van Wyck Expressway, Jamaica, NY 11418, USA.
Department of Rheumatology, Jamaica Hospital Medical Center, 8900 Van Wyck Expressway, Jamaica, NY 11418, USA.
Case Rep Rheumatol. 2019 Jul 24;2019:6173276. doi: 10.1155/2019/6173276. eCollection 2019.
Acutely decompensated dilated cardiomyopathy in a middle-aged patient without the typical risk factor profile presents a clinical dilemma. While cardiomyopathy is a known aspect of systemic lupus erythematosus (SLE), initial clinical presentation as decompensated dilated cardiomyopathy (DCM) is exceedingly rare in the literature. We share the case of a 49-year-old African-American female with no past medical history who presented with overt heart failure of 4 weeks evolution. Workup showed acute onset decompensated dilated cardiomyopathy, with a serologic profile compatible with SLE. She responded well to immunosuppressive steroid therapy. Literature review for SLE presenting as dilated cardiomyopathy with acute heart failure revealed a paucity of clinical evidence and consensus. Therefore, a comprehensive review of case reports was undertaken. A total of 10 cases were identified. Patients were 90% female and averaged 31 years of age. Dyspnea was the most common clinical presentation, and dilated cardiomyopathy with severely compromised left ventricular function was universally appreciated. Clinical presentation to diagnosis averaged 2 weeks. Immunosuppressive therapy regimens were universally employed; however, the regimens varied significantly. High-dose steroid therapy was most commonly used, and clinical and functional recovery was reported in 90% of individual case reports. Within the limited evidence and experience of therapeutic approaches, the efficacy of different singular or combined therapy is based solely on anecdotal case reports. Given the near-complete response to a short course of high-dose steroid therapy as much in the clinical recovery as in the resolution of DCM, the limited evidence based on review of these observational case studies and series supports the initial use of high-dose steroid therapy in acute lupus myocarditis.
一名无典型危险因素的中年患者出现急性失代偿性扩张型心肌病，这带来了临床难题。虽然心肌病是系统性红斑狼疮（SLE）的一个已知方面，但文献中以失代偿性扩张型心肌病（DCM）为初始临床表现极为罕见。我们分享一例49岁非裔美国女性病例，她既往无病史，因4周进展的明显心力衰竭前来就诊。检查显示急性起病的失代偿性扩张型心肌病，血清学特征与SLE相符。她对免疫抑制性类固醇治疗反应良好。对以急性心力衰竭的扩张型心肌病形式出现的SLE进行文献综述发现临床证据和共识匮乏。因此，对病例报告进行了全面回顾。共确定了10例病例。患者90%为女性，平均年龄31岁。呼吸困难是最常见的临床表现，普遍存在左心室功能严重受损的扩张型心肌病。从临床表现到诊断平均为2周。普遍采用免疫抑制治疗方案；然而，这些方案差异很大。最常用高剂量类固醇治疗，90%的个案报告中报道了临床和功能恢复情况。在治疗方法的有限证据和经验范围内，不同单一或联合治疗的疗效仅基于轶事性病例报告。鉴于短期高剂量类固醇治疗在临床恢复以及DCM消退方面的近乎完全反应，基于对这些观察性病例研究和系列的综述得出的有限证据支持在急性狼疮性心肌炎中初始使用高剂量类固醇治疗。
