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Systemic Sclerosis Less Severe in Children Than in Adults

Internal organ involvement less common in juvenile form of the disease

FRIDAY, Dec. 22 (HealthDay News) -- Systemic sclerosis may affect children less severely than adults because internal organs are less commonly affected in pediatric patients, according to the results of an international study that is the largest to date of such patients. The findings are published in the December issue of Arthritis & Rheumatism.

Giorgia Martini, M.D., Ph.D., of the University of Padua in Italy, and colleagues studied data on 153 juvenile systemic sclerosis patients in 55 facilities across North and South America, Europe and Asia.

The researchers found Raynaud's phenomenon was most common in the patients, followed by skin induration, which was seen in three-quarters of patients. One-third of patients had musculoskeletal symptoms and respiratory and gastrointestinal organs were the most commonly affected internal organs. Kidney, brain and cardiovascular involvement was rare.

Eighty-one percent of patients tested positive for antinuclear antibodies; 34 percent had antitopoisomerase I and 7.1 percent had anticentromere antibodies. Deceased patients' cardiovascular, respiratory and gastrointestinal systems were affected more often and earlier than those of survivors. Juvenile disease seemed less severe than the adult form.

"This study provides information on the largest collection of patients with juvenile systemic sclerosis ever reported," the authors write. "Juvenile systemic sclerosis appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome."

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