Cardiogenic Shock as the Initial Presentation of Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Baquero, Giselle A.; Banchs, Javier E.; Naccarelli, Gerald V.; González, Mario D.; Wolbrette, Deborah L.

doi:10.1111/j.1751-7133.2011.00283.x

Cited by 12 publications

(5 citation statements)

References 33 publications

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“…[18] There are few case reports of acute myocarditis and heart failure as an initial SLE presentation. The therapeutic approach for myocarditis starts from standard measures with supportive care as first-line therapy, whereas anti-heart failure medications and additional treatment are prescribed on the basis of underlying etiology [19] African-American patients are at increased risk for development of myocarditis [20], In more recent post-mortem studies, after the wide use of corticosteroids in lupus therapeutics, its prevalence was estimated as 0-8%.…”

Section: Discussionmentioning

confidence: 99%

Peculiarity of Systemic Lupus Erythematosus with Cardiac Involvement in Case Report and Literature Review

El Abasse

2024

JCCI

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Systemic lupus erythematosus (SLE, or lupus) is a condition in which the immune system attacks healthy cells and tissues throughout the body, it is characterized by aberrant activity of the immune system, leading to variable clinical symptoms. Case: A Moroccan 54 years old women presented a progressive dyspnea associated with palpitations with a history of hyperthyroidism, a pericardial effusion of great abundance punctured, the remainder of the diagnostic workup had revealed lupus erythematosus. The echocardiography found a global hypocontractility with an ejection fraction at 25%, a dilated atrial mass and mitral regurgtitaion, moderate aortic regurgitation with an image of vegetation on the non-coronary cusp, right ventricular dysfunction. Laboratory investigations revealed bycitopenia with high inflammatory markers, a renal dysfunction test result. Immunological tests showed a high positive antinuclear antibodies ans DNA antibodies. While hospitalization the patient presented a constrictive chest pain , the electrocardiogram showed an st segment elevation in the inferior derivation in relation with a coronary spasm.Three days later the patient presented a sudden neurological deficit type of right hemiparesis with facial paralysis and aphasia, the emergency MRI objectified a cortical frontotemporal stroke, in the acute phase, patient was thrombolysed by metalysis , finally the diagnosis of vasculitis of the central nervous system (CNS) was retained with ischemic and hemorrhagic lesions in favor of lupus vasculitis of the CNS . Discussion: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that may affect several different organs(5) , the underlying pathophysiologic mechanism remains unknown, new autoantibodies, such as antibodies to DNA , antibodies to extractable nuclear antigens , and anticardiolipin antibodies, were recognized as causative of clinical subsets of lupus(5) SLE is an unpredictable disease, it is characterized by alternating periods of remission and exacerbation and by a wide variety of clinical manifestations that may affect any organ. The case of our patient with SLE involved: cardiac type of lupus myopericadtitis in biventricular dysfunction with LVEF: 25%, , Normochromic normocytic anemia with positive coombs test and lymphopenia and thrombocytopenia ; lupus nephropathy satde IV ; Cerebral vasculitis. Cytopenias are common in patients with lupus, and moderate to severe lymphopenia is associated with high disease activity and organ damage. Up to 50% of patients with SLE have evidence of renal disease at presentation (10) Lupus end-stage kidney disease is associated with worse survival among dialysis and transplant patients. The risk for ischemic stroke is higher in patient with SLE. Stroke affects 3–20% of patients with SLE and usually occurs within the first 5 years of the diagnosis(13,14). The main Cardiovascular diseases of SLE are valvular heart diseases associated with Libman-Sacks disease lesions, sterile vegetations, serositis associated with pericardial disease. African-American patients are at increased risk for development of myocarditis(20); low ejection fraction is detected in the majority of the patients (25)Our patient had an ejection fraction around 25% . The most characteristic valvulopathy of SLE is Libman- Sacks endocarditis (LSE), which mainly affects the left cardiac valves (22) . Non-steroidal anti-inflammatory drugs (NSAIDs) are considered as the first choice in mild cases with minimal pericardial effusion (28) Corticosteroids should be used in refractory cases with or without antimalarials. Corticosteroids in combination with immunosuppressives (25) Cyclophosphamide and intravenous immunoglobulins (IVIGs) have been shown to improve cardiac function and mortality (30), in addition to the medical treatment of Heart failure. Conclusion: Diagnosis of SLE can be challenging and while several classification criteria have been posed, their utility in the clinical setting is still a matter of debate. Despite several agents shown to be efficacious in the treatment of SLE, the disease still poses significant morbidity and mortality risk in patients.

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Section: Discussionmentioning

confidence: 99%

Peculiarity of Systemic Lupus Erythematosus with Cardiac Involvement in Case Report and Literature Review

El Abasse

2024

JCCI

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“…There is a paucity of data on optimal treatment strategies for cardiogenic shock secondary to SLE myocarditis as current guidance is based upon consensus driven by case studies and observational data. Immunosuppression, usually with high-dose steroids is the cornerstone of treatment, although cyclophosphamide, azathioprine, mycophenolate, and intravenous immunoglobulin can be alternative options to control disease activity based on review of recent case reports described in literature [ 2 , 3 ]. Cyclophosphamide was utilized as an agent for steroid-sparing therapy given that it has been commonly used in SLE nephritis, severe cutaneous SLE, and other reported cases of myocarditis due to its favorable side effect profile and his Asian ethnicity [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Typical presenting SLE features include constitutional symptoms, cutaneous involvement, alopecia, oral ulcers, arthritis, renal involvement, and common cardiac symptoms, including chest pain, dyspnea, palpitations, and lower extremity swelling [ 1 ]. Cardiac manifestations include conduction disturbances, ischemia, cardiomyopathy, pericarditis, and myocarditis; however, rapid progression leading to cardiogenic shock is rare [ 2 ]. Clinical myocarditis occurs in nearly 5–10% of SLE patients, although post-mortem studies suggest a higher prevalence of subclinical myocarditis than previously known [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

SLE strikes the heart! A rare presentation of SLE myocarditis presenting as cardiogenic shock

Raval

Ruiz

Heywood

et al. 2021

BMC Cardiovasc Disord

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Background Although systemic lupus erythematosus (SLE) can affect the cardiovascular system in many ways with diverse presentations, a severe cardiogenic shock secondary to SLE myocarditis is infrequently described in the medical literature. Variable presenting features of SLE myocarditis can also make the diagnosis challenging. This case report will allow learners to consider SLE myocarditis in the differential and appreciate the diagnostic uncertainty. Case presentation A 20-year-old Filipino male presented with acute dyspnea, pleuritic chest pain, fevers, and diffuse rash after being diagnosed with SLE six months ago and treated with hydroxychloroquine. Labs were notable for leukopenia, non-nephrotic range proteinuria, elevated cardiac biomarkers, inflammatory markers, low complements, and serologies suggestive of active SLE. Broad-spectrum IV antibiotics and corticosteroids were initiated for sepsis and SLE activity. Blood cultures were positive for MSSA with likely skin source. An electrocardiogram showed diffuse ST-segment elevations without ischemic changes. CT chest demonstrated bilateral pleural and pericardial effusions with dense consolidations. Transthoracic and transesophageal echocardiogram demonstrated reduced left ventricular ejection fraction (LVEF) 45% with no valvular pathology suggestive of endocarditis. Although MSSA bacteremia resolved, the patient rapidly developed cardiopulmonary decline with a repeat echocardiogram demonstrating LVEF < 10%. A Cardiac MRI was a nondiagnostic study to elucidate an etiology of decompensation given inability to perform late gadolinium enhancement. Later, cardiac catheterization revealed normal cardiac output with non-obstructive coronary artery disease. As there was no clear etiology explaining his dramatic heart failure, endomyocardial biopsy was obtained demonstrating diffuse myofiber degeneration and inflammation. These pathological findings, in addition to skin biopsy demonstrating lichenoid dermatitis with a granular “full house” pattern was most consistent with SLE myocarditis. Furthermore, aggressive SLE-directed therapy demonstrated near full recovery of his heart failure. Conclusion Although myocarditis during SLE flare is a well-described cardiac manifestation, progression to cardiogenic shock is infrequent and fatal. As such, SLE myocarditis should be promptly considered. Given the heterogenous presentation of SLE, combination of serologic evaluation, advanced imaging, and myocardial biopsies can be helpful when diagnostic uncertainty exists. Our case highlights diagnostic methods and clinical course of a de novo presentation of cardiogenic shock from SLE myocarditis, then rapid improvement.

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“…In this case, we presumed the diagnosis of lupus myocarditis as she fulfilled the criteria for SLE, with most of the viral studies being negative and with no risk factors for atherosclerosis causing ischemic heart disease. Most of such cases were diagnosed on similar basis in the literature [21][22][23][24][25]. Treatment of lupus myocarditis has not been assessed in controlled trials since this is an infrequent manifestation of lupus.…”

Section: Discussion and Evaluationmentioning

confidence: 99%

Systemic lupus erythematosus presenting with status epilepticus and acute cardiomyopathy with acute heart failure: case report

Herath

Kulatunga

2020

Egypt J Neurol Psychiatry Neurosurg

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Introduction: Systemic lupus erythematosus is a connective tissue disorder, which causes complex multi organ involvement. Neurological and cardiac manifestations have been well noted but complications such as status epilepticus and acute myocarditis with heart failure at presentation remains uncommon. Case description: A 15-year-old, previously healthy, South Asian, Sri Lankan female presented with status epilepticus and the seizures only responded to intravenous midazolam and thiopentone sodium. On the fourth day, she developed tachycardia and shortness of breath and was found to have cardiomyopathy with heart failure with an ejection fraction 40%. Along with a positive urinary sediment, a positive ANA with a very high level of ds-DNA and low C3 and C4 levels confirmed our suspicion of systemic lupus erythematosus. Discussion and evaluation: Systemic lupus erythematosus presents in a variety of clinical presentations and the spectrum may range from unique to ubiquitous. Clinicians should have a high index of suspicion specially when encountering atypical presentations with multi-organ involvement, especially when patients tend to be young females. Status epilepticus and myocarditis are uncommon manifestations of systemic lupus erythematosus, and should be appreciated early, as if inappropriately managed would have a deleterious impact on mortality and morbidity.

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Cardiogenic Shock as the Initial Presentation of Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Cited by 12 publications

References 33 publications

Peculiarity of Systemic Lupus Erythematosus with Cardiac Involvement in Case Report and Literature Review

Peculiarity of Systemic Lupus Erythematosus with Cardiac Involvement in Case Report and Literature Review

SLE strikes the heart! A rare presentation of SLE myocarditis presenting as cardiogenic shock

Systemic lupus erythematosus presenting with status epilepticus and acute cardiomyopathy with acute heart failure: case report

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