OJPed  Vol.2 No.2 , June 2012
First report of cardiac tamponade in pediatric-onset mixed connective tissue disease
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.
Cite this paper: Gupta, R. , Marks, M. , Spalding, S. and Qureshi, A. (2012) First report of cardiac tamponade in pediatric-onset mixed connective tissue disease. Open Journal of Pediatrics, 2, 165-169. doi: 10.4236/ojped.2012.22027.

[1]   Sharp, G.C., Irvin, W.S., Tan, E.M., Gould, R.G. and Holman, H.R. (1972) Mixed connective tissue disease— An apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). American Journal of Medicine, 52, 148-159. doi:10.1016/0002-9343(72)90064-2

[2]   Singsen, B.H., Bernstein, B.H., Kornreich, H.K., King, K.K., Hanson, V. and Tan, E.M. (1977) Mixed connective tissue disease in childhood: A clinical and serologic survey. Journal of Pediatrics, 90, 893-900. doi:10.1016/S0022-3476(77)80555-6

[3]   Kasukawa, R., Tojo, T. and Miyawaki, S. (1987) Mixed connective tissue disease and antinuclear antibodies. Elsevier, Amsterdam, 41-47.

[4]   Mier, R.J., Shishov, M., Higgins, G.C., Rennebohm, R.M., Wortmann, D.W., Jerath, R. and Alhumoud, E. (2005) Pediatric-onset mixed connective tissue disease. Rheumatic Disease Clinics of North America, 31, 483-496. doi:10.1016/j.rdc.2005.04.002

[5]   Kumar, M.S., Smith, M. and Pischel, K.D. (2006) Case report and review of cardiac tamponade in mixed connective tissue disease. Arthritis & Rheumatism, 55, 826-830. doi:10.1002/art.22227

[6]   Bezerra, M.C., Saraiva, F., Carvalho, J.F., Caleiro, M.T., Goncalves, C.R. and Borba, E.F. (2004) Cardiac tamponade due to massive pericardial effusion in mixed connective tissue disease: Reversal with steroid therapy. Lupus, 13, 618-620. doi:10.1191/0961203303lu1043xx

[7]   Breda, L., Nozzi, M., De Sanctis, S. and Chiarelli, F. (2010) Laboratory tests in the diagnosis and follow-up of pediatric rheumatic diseases: An update. Seminars in Arthritis and Rheumatism, 40, 53-72. doi:10.1016/j.semarthrit.2008.12.001

[8]   Michels, H. (1997) Course of mixed connective tissue disease in children. Annals of Internal Medicine, 29, 359-364.

[9]   Burdt, M.A., Hoffman, R.W., Deutscher, S.L., Wang, G.S., Johnson, J.C. and Sharp, G.C. (1999) Long-term outcome in mixed connective tissue disease: Longitudinal clinical and serologic findings. Arthritis & Rheumatism, 42, 899-909. doi:10.1002/1529-0131(199905)42:5<899::AID-ANR8>3.0.CO;2-L

[10]   Alpert, M.A., Goldberg, S.H., Singsen, B.H., Durham, J.B., Sharp, G.C., Ahmad, M., Madigan, N.P., Hurst, D.P. and Sullivan, W.D. (1983) Cardiovascular manifestations of mixed connective tissue disease in adults. Circulation, 68, 1182-1193. doi:10.1161/01.CIR.68.6.1182

[11]   Oetgen, W.J., Mutter, M.L., Lawless, O.J. and Davia, J.E. (1983) Cardiac abnormalities in mixed connective tissue disease. Chest, 83, 185-188. doi:10.1378/chest.83.2.185

[12]   Roy, C.L., Minor, M.A., Brookhart, M.A. and Choudhry, N.K. (2007) Does this patient with a pericardial effusion have cardiac tamponade? Journal of the American Medical Association, 297, 1810-1818. doi:10.1001/jama.297.16.1810