Juvenile systemic sclerosis: report of three cases and review of Japanese published work.
Juvenile or childhood onset systemic sclerosis (SSc) is rare. In our department, we encountered three juvenile SSc patients among the overall 206 SSc patients. All three cases were diffuse cutaneous type SSc (dSSc) with positive antinuclear antibodies. In this report, we report the three patients and analyze clinical and serological features of 58 Japanese patients with juvenile SSc. Forty-eight patients (92.3%) were classified as dSSc and 21 out of 35 patients (60%) had anti-topoisomelase I antibodies, while anticentromere antibodies were not detected in any patients. Prevalence of lung fibrosis and scleroderma renal crisis was low, however, the rate of cardiac involvement was higher than that in adult patients.
Aoyama
Kumi
K
Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. kumako@ma.neweb.ne.jp
Nagai
Yayoi
Y
Endo
Yukie
Y
Ishikawa
Osamu
O
D000293:Adolescent; D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D002503:Centromere / Q000276:immunology; D002648:Child; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D005260:Female; D006331:Heart Diseases / Q000150:complications; D006801:Humans; D007674:Kidney Diseases / Q000150:complications; D011658:Pulmonary Fibrosis / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology* / Q000473:pathology; D012867:Skin / Q000473:pathology*
D000974:Antibodies, Antinuclear; D004264:DNA Topoisomerases, Type I