Juvenile dermatomyositis

A clinical and immunologic study

Lauren M Pachman*, Nora Cooke

*Corresponding author for this work

Research output: Contribution to journalArticle

106 Citations (Scopus)

Abstract

Twenty-one children were diagnosed as having juvenile dermatomyositis on the basis of the strict criteria of Bohan and Peter. In addition to the typical skin and muscle changes, abnormalities of esophageal motility (eight of 19), pulmonary function (14 of 17), ECG (10 of 20), and gastrointestinal absorption of D-xylose (two of eight) with active disease were observed. Clinical signs of other collagen vascular disease appeared in five children. Serologic evaluation demonstrated that ANA and rheumatoid factor were transiently positive in six; one child developed a persistently positive rheumatoid factor after four years of disease inactivity. Antibody to ENA was negative in all, but antibody to PM-1 antigen was present in four of 18. Six had a low C3 or C4; evidence of immune complexes was demonstrated by Clq or Raji binding in eight with active disease. One child was IgA deficient. The HLA-B8 antigen was present in 72% of the Caucasion children as compared with the expected incidence of 21%. Therefore, classical dermatomyositis in children has more systemic involvement then previously appreciated, may be related to the presence of circulating immune complexes, and appears to be under immunogenetic control.

Original languageEnglish (US)
Pages (from-to)226-234
Number of pages9
JournalThe Journal of pediatrics
Volume96
Issue number2
DOIs
StatePublished - Jan 1 1980

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Rheumatoid Factor
Antigen-Antibody Complex
HLA-B8 Antigen
Immunogenetics
Collagen Diseases
Dermatomyositis
Antibodies
Xylose
Vascular Diseases
Immunoglobulin A
Juvenile dermatomyositis
Clinical Studies
Electrocardiography
Antigens
Muscles
Lung
Skin
Incidence
Gastrointestinal Absorption

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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abstract = "Twenty-one children were diagnosed as having juvenile dermatomyositis on the basis of the strict criteria of Bohan and Peter. In addition to the typical skin and muscle changes, abnormalities of esophageal motility (eight of 19), pulmonary function (14 of 17), ECG (10 of 20), and gastrointestinal absorption of D-xylose (two of eight) with active disease were observed. Clinical signs of other collagen vascular disease appeared in five children. Serologic evaluation demonstrated that ANA and rheumatoid factor were transiently positive in six; one child developed a persistently positive rheumatoid factor after four years of disease inactivity. Antibody to ENA was negative in all, but antibody to PM-1 antigen was present in four of 18. Six had a low C3 or C4; evidence of immune complexes was demonstrated by Clq or Raji binding in eight with active disease. One child was IgA deficient. The HLA-B8 antigen was present in 72{\%} of the Caucasion children as compared with the expected incidence of 21{\%}. Therefore, classical dermatomyositis in children has more systemic involvement then previously appreciated, may be related to the presence of circulating immune complexes, and appears to be under immunogenetic control.",
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Juvenile dermatomyositis : A clinical and immunologic study. / Pachman, Lauren M; Cooke, Nora.

In: The Journal of pediatrics, Vol. 96, No. 2, 01.01.1980, p. 226-234.

Research output: Contribution to journalArticle

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