The value of humoral immune phenomena in children and adolescents with circumscribed scleroderma

1989 
: Sera taken from 35 children and teen-agers with different clinic variants of the circumscribed scleroderma were investigated for the presence of FANA, anti-DNA-, anti-DNP-, anti-ENA-(extractable nuclear antigens)-, and anti-Ro/SSA-antibodies. In 85.7% of the patients, humoral immune phenomena were ascertainable. Whereas FANA above all in patients with clinically rapidly progredient running linear form (71%) were present, anti-dsDNA- and anti-DNP-antibodies may be worth as hint upon a visceral manifestation. Therefore, they may be valid as leading antibodies for a subset of the circumscribed scleroderma, which is being designated by extracutaneous participation and the skin mostly exhibit a linear variant. The frequency of the evidence of the characteristic immune phenomena in patients with circumscribed scleroderma, which are typically in systemic lupus erythematosus, is considered as hint to the immune pathogenesis of the disease. The functional defects of the kidneys, catched with the isotope-clearance refer to a possible systemic character of the disease-process. Humoral immune phenomena are suitable for differentiation of the cutaneous and extracutaneous progress forms, in which high titer of FANA with homogeneous pattern of fluorescence, anti-dsDNA- and anti-DNP-antibodies have a special importance. Compared to it anti-ssDNA-antibodies and anti-Ro/SSA-antibodies characterize the high degree of activity of cutaneous and extracutaneous progress forms.
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