Pericardial Effusion Associated with Left Ventricle Hypertrophy and Macrophage Activation Syndrome Revealing Systemic Lupus Erythematosus: A Case Report

Introduction: Systemic lupus erythematosus is an autoimmune disease of unknown etiology. Despite the rarity of clinical manifestations, cardiac involvement is one of the major causes of mortality.

Case Presentation: We report the case of a patient with severe pericardial effusion and concentric left ventricle hypertrophy (LVH) in whom lupus was manifested by macrophage activation syndrome. she was admitted to the emergence department for fever, chest pain and progressive dyspnea. She also reports having developed inflammatory polyarthralgia for 1 year. Cardiomegaly was noted on chest radiograph. The ECG showed microvoltage and sinus tachycardia. The echocardiography showed a severe pericardial effusion with diastolic collapse of the right ventricle and respiratory variations. Faced with this brutal scenario (pre- tamponade), the patient underwent pericardiocentesis with extraction of 900cc of citrine-yellow exudative fluid. A macrophage activation syndrome test was carried out coming back positive. The diagnosis of systemic lupus erythematosus complicated by macrophage activation syndrome was retained (according to the ACR/EULAR 2019 criteria). The patient was treated by corticotherapy. The evolution was favorable with disappearance of the pericardial effusion. This therapy prevents recurrence of symptoms.

Discussion: Cardiovascular manifestations of systemic lupus erythematosus may involve all heart structure specially pericardium, valve, conduction system and coronary arteries. Pericardial involvement is the first to occur in 11% to 54% of cases according to some studies. Patients with systemic lupus erythematosus have an increased prevalence of left ventricle hypertrophy. The studies suggest that inflammation-mediated arterial stiffening is likely to be the underlying mechanism of left ventricle hypertrophy in systemic lupus erythematosus. Thus, the occurrence of macrophage activation syndrome at the same time as lupus appears to be a rare but seems to define a severe form of systemic lupus erythematosus. Early therapy with high-dose intravenous corticosteroids is usually used in macrophage activation syndrome of autoimmune origin.

Conclusions: Systemic lupus erythematosus revealed by an activation syndrome as well as increased LV mass present two progressive indicators of cardiac morbidity and mortality requiring targeted and early treatment.