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The British Journal of Rheumatology, Vol 37, 222-227, Copyright © 1998 by British Society for Rheumatology

ORIGINAL PAPERS

Atypical juvenile generalized scleroderma presenting as polyarthritis and failure to thrive

NM Wulffraat, P Woo, M Rooney, K De Meer and W Kuis
University Hospital for Children het Wilhelmina kinderziekenhuis, Utrecht, The Netherlands.

In this Grand Round, two children are described with atypical generalized scleroderma and severe failure to thrive. Neither had Raynaud's phenomenon nor evidence of gastrointestinal (GI) disease. Treatment with non-steroidal anti-inflammatory drugs, prednisone, D- penicillamine, alpha and gamma interferon was unsuccessful in reversing the sclerodermatous changes and growth arrest. Dietary intake analysis and extensive GI investigation were performed in both. In one case, resting energy expenditure (Ee) was repeatedly measured. His intake did not meet requirements for growth. Supplemental tube feeding (900 kcal in 6 h) was commenced, causing an increase in weight from 11 to 16 kg. The other patient refused supplementary tube feeding and no weight gain has been observed for 5 yr. In conclusion, early-onset generalized scleroderma in the absence of visceral involvement, but with growth failure, may represent an atypical form of systemic sclerosis. The response of the two patients to conventional therapy was disappointing. However, the rapid catch-up growth induced by tube feeding observed in one patient underlines the importance of adequate dietary management.
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