Systemic Lupus Erythematosus Presenting as Myopericarditis with Acute Heart Failure: A Case Report and Literature Review

Abstract

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.

Mycophenolate Mofetil Use in Severe Myocarditis Complicating Systemic Lupus

“…Myocarditis remains rare but life-threatening with significant morbidity and mortality in 50% of cases, especially when it is present as an initial manifestation [5]. Few cases have presented with myocarditis complicated with cardiogenic shock associated with systemic lupus [2,[4][5][6][7]. Pleural and pericardial effusion occur in a large number of patients with myocarditis, and this was the case of our patient too.…”

Perimyocarditis as First Manifestation of Systemic Lupus Erythematosus Successfully Treated With Heart Failure and Immunosuppressive Therapy

“…Since there are no guidelines for the treatment of myocarditis in systemic rheumatic diseases, the treatment is based on the experience of individual rheumatologists and cardiologists, as well as review papers, case reports, and expert opinions published in the literature. In our case, we were guided by the 2016 ESC guidelines for HF valid at that time, the Position statement of the ESC Working Group on Myocardial and Pericardial Disease, EULAR recommendations for the management of SLE, previously reported case reports, and reviews [8][9][10][11][12][13][14][15]. According to the literature, treatment of lupus myocarditis requires an initial period of intensive immunosuppression to decrease disease activity, followed by a longer period of less intensive therapy to consolidate the response and prevent relapses [9,12].…”

Perimyocarditis as First Manifestation of Systemic Lupus Erythematosus Successfully Treated with Heart Failure and Immunosuppressive Therapy

“…In our case, we established a diagnosis of SLE according to the 2019 EULAR/ACR classification criteria and after the exclusion of other causes of myocarditis. In the treatment of myocarditis in SLE, we were guided by the 2016 ESC guidelines for HF valid at that time, the position statement of the ESC Working Group on Myocardial and Pericardial Disease, EULAR recommendations for the management of SLE, previously reported case reports, expert opinions, and reviews [8][9][10][11][12][13][14][15]. Most of the treatment evidence is derived from case reports, case-control, and cohort studies.…”