ANTINUCLEAR ANTIBODIES IN CHILDREN WITH LOCALIZED SCLERODERMA

Objective. To determine the prevalence and antigenic specificities of antinuclear antibodies (ANA) in children with localized scleroderma. M ethods, The ANA profiles of 27 children with localized scleroderma wer e determined. The study group comprised 21 children with linear sclero derma, 5 with morphea, and 1 with combined linear scleroderma and morp hea. Sera were evaluated for the presence of ANA by indirect immunoflu orescence and for reactivity with specific nuclear antigens by ELISA a nd immunoblotting. Results, Seventeen patients (63 %) had positive tes ts for ANA. Of these sera 10 displayed a finely speckled pattern, 5 a combined nucleolar and finely speckled nuclear pattern, and 2 a nucleo lar pattern only. Fourteen of 21 (67 %) with linear scleroderma were A NA positive, Three of 5 patients with morphea (60 %) had ANA. The 1 pa tient with both linear scleroderma and morphea was ANA negative. Fifte en sera (56 %) contained antibodies to denatured DNA (dDNA). Eleven se ra (41 %) had antibodies to one or more high mobility group (HMG) prot eins, 4 (15%) reacted with one or more histones and 1 serum (4%) react ed with topoisomerase I (Scl-70). Conclusion. ANA are present in most children with localized scleroderma and frequently have specificity fo r dDNA and HMG proteins. Children with localized scleroderma, like pat ients with systemic sclerosis (SSc), commonly have ANA and antibodies to dDNA. Unlike patients with SSc, however, childhood localized sclero derma is uncommonly associated with antibodies to certain specific nuc lear and nucleolar constituents that typically occur in association wi th SSc.