Mixed connective tissue disease in childhood. Relationship Sjogren's syndrome
A. Fraga, J. Gudino, F. Ramos-Niembro and D. Aiarcon-Segovia
Mixed connective tissue disease (MCTD) seems to be a distinct entity that
has some manifestations of systemic lupus erythematosus, scleroderma,
polymyositis, and Sjogren's syndrome and is serologically characterized by
the presence of an antibody to ribonucleoprotein. We report the cases of
three children with MCTD with high titers of antibody to ribonucleoprotein.
Two fulfilled criteria of lupus erythematosus, two had polymyosis; all
three had suggestive features of scleroderma, fulfilled criteria for the
diagnosis of juvenile rheumatoid arthritis, and had Sjogren's syndrome.
Additional superimposed features of another connective tissue disease
should arouse suspicion of MCTD. All three of our patients responded
adequately to corticosteroid treatment that makes recognition of this
entity by the pediatrician all the more important.
