CAR-12. When a case gets under your skin.

The purposes of the study were to explore the differential diagnosis of massive pericardial effusion; to discuss common progression of scleroderma and scleroderma-like diseases; and to clarify the method and purpose behind serologically defining diseases with morphea. We report a 57-year-old African American male presenting with severe dyspnea and chest pain. Initial chest radiography (CXR) revealed a massive cardiac silhouette. Echocardiogram confirmed a significant pericardial effusion. Prior to this presentation, patient had never presented with cardiac or pulmonary disease. A review of literature found three case series that explored the etiology of large pericardial effusions. One possibility suggested was a connective tissue disease and laboratory screening revealed high titer rheumatoid factor and anti-nuclear antibody with significant elevation in erythrocyte sedimentation rate. Pericardial effusion was treated with drainage, and since the patient's symptoms were completely relieved, no other therapy was pursued. Several weeks later the patient presented again with severe dyspnea and abnormal skin manifestations on physical exam. CXR did not show cardiomegaly and echocardiogram ruled out recurrent pericardial disease. Examination revealed rapidly evolving morphea characteristic of a scleroderma-like disease. CT imaging demonstrated a progressive fibrosing disease of the lungs. Pulmonary function tests confirmed a severe restrictive physiology. Laboratory data defined patient's disease as a systemic sclerosing connective tissue disorder with positive Smith (Sm) and U3 RNP (fibrillarin) antibodies, but negative scleroderma (Scl-70) and anti-centromere antibodies. The differential diagnosis of massive pericardial effusion is broad and diverse. We report massive pericardial effusion as the initial presentation of a systemic sclerosing disorder. Particular challenges in this case were the specific classification of the patient's disorder, which was able to guide treatment and prognostic evaluation.

Kevin Luce, MD, and Mukta Panda, MD. University of Tennessee-Chattanooga Unit, Chattanooga, TN.