Familial aggregation of autoimmune disease in juvenile dermatomyositis

Niewold, T. B.; Wu, S. C.; Smith, M.; Morgan, G. A.; Pachman, L. M.

Pediatrics. 2011 Apr 20; 127(5):e1239-46


OBJECTIVE: Familial aggregation of autoimmune diseases likely reflects shared pathogenic factors between different diseases. Familial aggregation of autoimmunity has not been examined in juvenile dermatomyositis. Interferon-alpha is thought to be a pathogenic factor in both systemic lupus erythematosus and juvenile dermatomyositis, and we have previously demonstrated familial aggregation of serum interferon-alpha. METHODS: Family histories were obtained from 304 families of children with juvenile dermatomyositis via 3-generation structured interviews performed by the same person. Rates of autoimmune disease in families of children with juvenile dermatomyositis were compared with published population rates. Serum interferon-alpha, tumor necrosis factor-alpha, and neopterin were measured using standard techniques. RESULTS: A total of 51% of families of children with juvenile dermatomyositis reported at least 1 additional member affected by an autoimmune disease. In particular, both type 1 diabetes and systemic lupus erythematosus were significantly more common than would be expected (odds ratio >5, P

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