Systemic sclerosis with pulmonary involvement and right ventricular failure in a child.

Abstract

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic 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, treatment, and outcome.

• Authors: C D Liang, S F Ko, C B Huang, C K Niu
• Journal: Journal of the Formosan Medical Association = Taiwan yi zhi (J Formos Med Assoc) Vol. 96 Issue 11 Pg. 917-20 (Nov 1997) ISSN: 0929-6646 [Print] Singapore
• PMID: 9409127 (Publication Type: Case Reports, Journal Article)

Topics

• Child
• Female
• Humans
• Hypertension, Pulmonary (etiology)
• Hypertrophy, Right Ventricular (etiology)
• Pulmonary Fibrosis (etiology)
• Scleroderma, Systemic (complications)