Systemic sclerosis in children: a national retrospective survey in Japan

A retrospective questionnaire survey of pediatric departments, for childhood collagen disease from 1985 to 1994 was used to clarify the clinical features of scleroderma in Japan. In the primary survey, 0.9% of the children with a rheumatic condition and scleroderma. Answers to this questionnaire were received on 18 (localized 9; systemic 9) patients from 15 institutions. In order to examine systemic sclerosis (SSc), seven cases of SSc in Japanese articles during the same period as the questionnaire were added to these answers and compared to the Japanese epidemic study investigated by Fukuyama in 1974. There were 16 children, seven boys and nine girls, with SSc during the 10-year period in Japan. The mean age of onset of symptoms was 8.0 +/- 2.8 years and the age at diagnosis 10.1 +/- 3.0 years. Eighty percent of children had Raynaud's phenomenon at the onset of SSc, and skin and musculoskeletal involvement was highly recognized during the course of the disease. Atrophy of the frenulum linguae and lung fibrosis were commonly seen in SSc. In serological studies, 80% of children have antinuclear antibodies and approximately 50% of patients have anti-Scl-70 (topoisomelase I) antibodies at the onset and during the course of childhood SSc. The prognosis is poor, as remission occurred in only one child. The clinical symptoms and examination of serological autoimmune antibodies were supportive of an early diagnosis of SSc. When compared to the previous national survey of children with SSc, the present results showed that the male-to-female ratio was reduced, the age at onset was low, the positive incidence of serological autoimmune antibodies elevated, and the usage of vasodilators and nonsteroid anti-inflammatory drugs (NSAID) increased, with corticosteroids decreased. But, the positive percentage of clinical symptoms were not changed in both studies. For a complete retrospective nationwide epidemic survey carried out on children with scleroderma, especially SSc, it is important to include dermatology departments.