A 22-year-old woman, without any formerly diagnosed diseases, presented to the emergency department with a 3 day history of dyspnea, fatigue and central chest pain. She reported increasing dyspnea that developed in the previous month. She also had intermittent pain in the small joints for several months. There was no family history of SLE. On examination, she had a temperature of 38.2 C, a heart rate of 115 beats/minute, blood pressure of 90/60mmHg, respiratory rate of 25 breaths/minute, muffled heart sounds and jugular distention. The electrocardiogram showed sinus tachycardia and low voltage. The chest radiograph showed an enlargement of the cardiac silhouette with a right-sided pulmonary effusion (Figure 1). The echocardiography revealed a large circumferential pericardial effusion (Figure 2), with diastolic collapse of the right atrium, dilated inferior vena cava and 30% respiratory variation of the Doppler mitral valve, confirming the diagnosis of cardiac tamponade. Initial workup showed normochromic normocytic anemia with hemoglobin at 8.5 g/dl, CRP: 75 mg/L, renal and liver function tests were normal.
Emergency pericardiocentesis was indicated but could not be performed because of the incapability to place the guidewire into the pericardial cavity. So, urgent surgical pericardiectomy with window procedure was realized, 1200 of clear fluid was evacuated. The pericardial fluid contained 2900 white blood cells/mm3, 100 red blood cells/mm3, bacteriological culture and cytologic examinations yielded negative results. The pericardial biopsy showed features of nonspecific inflammation, malignancy and tuberculosis were ruled out. Further investigations were accomplished to determine the etiology. Immunological workup revealed antinuclear antibodies titer of 1: 1280, positive anti-SSA and anti-SM antibodies and hypocomplementemia. The coombs test was positive. The diagnosis of systemic lupus erythematosus was established based on hemolytic anemia, serositis, arthralgia, positive anti nuclear and anti-SM antibodies and low complement. The patient was started on intravenous therapy with methylprednisolone followed by prednisone 60mg by mouth daily with hydroxychloroquine. She had a good clinical response and control echocardiography showed complete resolution of the pericardial effusion without recurrence.