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

Abstract

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.