ABSTRACT Mixed Connective Tissue Disease (MCTD) is relatively rare in children and typically presents with constitutional symptoms, rash, Raynaud’s phenomenon, and musculoskeletal symptoms. Cardiac involvement is an infrequent complication of MCTD usually occurring in the form of pericarditis without tamponade physiology. However, we present a case of a 10-year-old, previously healthy, African American male who developed pericarditis and tamponade as an initial manifestation of MCTD. One month prior to diagnosis, the child was hospitalized for fevers, knee pain and knee swelling. Arthrocentesis revealed leukocytosis yet no laboratory evidence of an infectious etiology. He was discharged on naproxen with a presumptive diagnosis of post-infectious arthritis. Over the next two weeks, the child was evaluated several times for intermittent, left-sided, chest pain. Electrocardiograms and chest radiographs were found to be normal. His non-steroidal anti-inflammatory medications were continued for supposed musculoskeletal chest pain. Ultimately the child was admitted for fever, chest pain and a pericardial effusion on echocardiogram. Within two days, symptoms progressed to include orthopnea and jugular venous distension. Pulsus paradoxus was demonstrable on exam and electrical alternans on cardiac monitor. Repeat echocardiogram revealed an increased effusion with tamponade physiology necessitating pericardiocentesis. Coincidentally, the patient began demonstrating Raynaud’s phenomenon and auto-antibodies supportive of MCTD returned positive. Symptoms improved on corticosteroids. This case illustrates the importance of considering an acute and critical process in an otherwise chronically evolving disease. It serves as the first report of such an occurrence in pediatriconset MCTD.
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