SYSTEMIC-SCLEROSIS WITH PULMONARY INVOLVEMENT AND RIGHT-VENTRICULAR FAILURE IN A CHILD

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmona ry interstitial fibrosis, pulmonary hypertension, and right ventricula r failure. The diagnosis was confirmed by skin manifestations, high re solution computed tomography, cardiac catheterization, and anti-nuclea r antibodies. Nifedipine, prednisolone, digoxin, and furosemide were g iven. There was remission of the right ventricular failure and dyspnea , and the skin showed partial improvement. The patient remained asympt omatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, trea tment, and outcome.