Juvenile dermatomyositis: immunogenetics, pathophysiology, and disease expression.

Data are rapidly accumulating documenting disease susceptibility may be linked to genetic markers (DQA1*0501 or related alleles) which are associated with upregulation of IFN-alpha/beta inducible genes elicited in response to an antigen which may be viral. Continued immune-mediated inflammation, resulting in partial lipodystrophy and pathological calcifications, may be fanned by the increased production of TNF alpha usually associated with the TNF alpha-308A allele.