Case Study: Rheumatic Disease Presents With Rare Cardiac Manifestation

Large pericardial effusion MRI
Case study of a woman with a rare cardiac manifestation of a rheumatic disease.


Systemic lupus erythematosus (SLE) is an autoimmune disease that can involve any organ system, leading to diverse clinical presentation. Pericardial involvement, typically a pericardial effusion, is the most common type of echocardiographic abnormality found in SLE, reported in up to 50% of patients with SLE, and is usually asymptomatic. Large and/or hemodynamically significant effusions resulting in cardiac tamponade are rare with 1% to 2.5% incidence in patients with SLE.1 Cardiac tamponade is a medical emergency that develops when a pericardial effusion reaches a critical amount, limiting cardiac inflow and affecting cardiac muscle movement, which may lead to hemodynamic compromise.


A 27-year-old African American woman presented to the emergency department, with complaints of shortness of breath, fever, and upper abdominal pain for 4 days. The shortness of breath was progressive, worse with supine position, and improved by leaning forward. The patient described the abdominal pain as sharp and nonradiating, and associated with nausea and episodes of emesis. Systemic review was significant for intermittent fever, hair loss, chronic body aches, and arthralgia affecting the wrist, elbow, and knee joints. She denied chest pain, palpitation, syncope, seizures, oral ulcers, or a rash. The patient had a past medical history of type 1 diabetes, major depressive disorder, and hypertension. She had no known family history of autoimmune disease.

On examination, she had a heart rate of 98 bpm, a respiratory rate of 18 breaths/minute, blood pressure of 125/82 mmHg, and a temperature of 98.3 °F. She was considered overweight, with a body mass index of 29.01, had signs of muscle loss, and edema. She maintained oxygen saturation 98% on room air. On cardiovascular examination, heart sounds were distant but normal; no murmurs or cardiac rub was observed. There was no evidence of pulsus paradoxus. Jugular venous distension was evident. Respiratory examination revealed clear lungs bilaterally. Abdominal exam was significant for epigastric tenderness. The rest of the examination was significant for lymphadenopathy. There was no evidence of mouth ulcers, skin rash, skin thickening, or joint inflammation.

Electrocardiography showed low voltage sinus tachycardia without electrical alternans. The chest radiograph displayed an enlarged cardiac silhouette with pulmonary vascular congestion. Due to intermittent fever and tachycardia during the hospital stay, the patient underwent computed tomography (CT) to rule out occult infection. The CT revealed large-volume pericardial effusion with evidence of pericarditis and evidence of anasarca. Echocardiography showed large pericardial effusion with invagination of right atrial wall, suggestive of early cardiac tamponade. Echocardiography-guided pericardiocentesis was then performed and 500 mL of pericardial fluid was aspirated. Repeat echocardiography showed complete resolution of the pericardial fluid with no additional reaccumulation, with normal cardiac chambers movement.

Laboratory investigations revealed microcytic anemia with leukopenia. Erythrocyte sedimentation rate (ESR) was markedly elevated at 106 mm/hour and C-reactive protein (CRP) was 17.6 mg/dL. Hemoglobin A1c was 6.3%. Renal parameters were acutely mildly elevated. Cardiac biomarkers and liver enzymes were normal. Urinalysis showed +2 proteinuria. The patient’s serum tested strongly positive for antinuclear antibodies (ANA) with homogeneous pattern; antidouble stranded DNA antibodies (anti-dsDNA) were found to be positive while anti-Smith autoantibodies were negative. Serum complement levels were variable with normal C3 of 22.1 mg/dL (normal range, 10-40 mg/dL) and low C4 of 64 mg/dL (normal range, 90-180 mg/dL). The 24-hour urine protein collection showed 1770 mg/24 hours. Pericardial fluid analysis was suggestive of exudative pericardial effusion, with neutrophilic prominence and elevated pericardial fluid protein; the pericardial fluid cytology showed mixed inflammatory cells without malignant cells.

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This article originally appeared on Rheumatology Advisor