ABSTRACT
Ninety children with definite juvenile dermatomyositis (JDMS), who had been HLA typed, were tested for the presence of tissue or organ-specific antibodies. Sixty had active disease at the time of study. The mean disease duration was 4 years, and 30 had soft tissue calcifications. The following autoantibodies were sought: thyroid, gastric parietal cells, smooth muscle, striated muscle, microsomes, mitochondria, DNA, extractable nuclear antigen, Sm, PM-1, antinuclear antibody (ANA), and rheumatoid factor. Only the ANA and PM-1 were more frequent in patients than in controls (P less than 0.0002 and P less than 0.001, respectively). Higher levels of immune complexes (P less than 0.01) were found in sera from patients with JDMS than in sera from controls and were correlated with the presence of ANA in patients (P less than 0.01). Soft tissue calcification was not associated with any autoantibody or HLA antigen, but with disease duration and activity (P less than 0.001 and P less than 0.05, respectively). There was no association between the occurrence of any autoantibody and the presence of HLA-B8 or DR3 among the white patients with JDMS. The frequency of autoantibodies in 43 full siblings of children with JDMS was not increased. We conclude that children with JDMS, with or without HLA-B8/DR3, do not show evidence of a generalized nonspecific antibody response to tissue antigens. The significance of the increased antibody to nuclear antigens ANA and PM-1 remains to be determined.
Subject(s)
Antibodies, Antinuclear/analysis , Autoantibodies/analysis , Dermatomyositis/immunology , Adolescent , Adult , Antigen-Antibody Complex/immunology , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Mitochondria/immunology , Muscles/immunology , Organ SpecificityABSTRACT
Typing for HLA-A and -B antigens was performed on 87 children with definite juvenile dermatomyositis (JDMS). A significantly increased frequency of HLA-B8 (estimated relative risk = 2.8, Pc less than 0.01) was observed among White patients, but not among Blacks or Latin Americans with JDMS. No abnormality of HLA haplotype segregation was observed among 38 healthy siblings of the JDMS probands.
Subject(s)
Dermatomyositis/immunology , HLA Antigens/genetics , Adolescent , Adult , Black People , Child , Child, Preschool , Dermatomyositis/genetics , Female , HLA-A Antigens , HLA-B Antigens , HLA-B8 Antigen , Humans , Infant , Latin America/ethnology , Male , White PeopleABSTRACT
Polyarteritis was diagnosed in three girls, 9 to 10 years old, by kidney and skin biopsies. They were treated with a combination of prednisone (1.5 to 2 mg/kg) and cyclophosphamide (2 mg/kg) for up to 12 months. The illness was severe in all three, complicated by hypertension, seizures, pulmonary infiltrates, renal failure, or hallucinations. All three patients are alive and well with no or minimal residual symptoms two to three years after therapy was discontinued. The treatment with corticosteroids or with a combination of steroids and immunosuppressive drugs seems to improve the prognosis of polyarteritis considerably.
