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Elevated serum interferon-α activity in juvenile dermatomyositis: Associations with disease activity at diagnosis and after thirty-six months of therapy
Arthritis & Rheumatism, 06/05/09

Niewold TB et al. – Study reports that serum IFN-α activity was associated with higher serum levels of muscle-derived enzymes and a shorter duration of untreated disease in patients with newly diagnosed juvenile dermatomyositis (DM) and was inversely correlated with measures of chronic disease activity at 36 mo postdiagnosis, suggesting that IFN-α could play a role in disease initiation in juvenile DM.

• Aim was to examine in children with juvenile DM:
  • serum IFN-α activity and
  • determine relationships between IFN-α and indicators of disease activity and severity
• 39 subjects; serum samples were obtained at the time of diagnosis from 18 untreated pts with juvenile DM
• Second samples from 11 of these pts were obtained at 24 mo, while they were receiving treatment
• Third samples were obtained from 7 of these pts at 36 mo
• Remaining 21 children were studied 36 mo after their initial diagnosis
• Serum IFN-α activity was measured using a functional reporter cell assay

• Pts with juvenile DM had higher serum IFN-α activity vs both pediatric and adult healthy control subjects
• In untreated pts, serum IFN-α activity was:
  • positively correlated with serum muscle enzyme levels and
  • inversely correlated with the duration of untreated disease
• TNF-α-308A allele was associated with higher serum IFN-α levels only in untreated pts
• At 36 mo, serum IFN-α levels were inversely correlated with:
  • muscle enzyme levels in those pts still requiring therapy and
  • with the skin DAS in those pts who had completed therapy

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