Treatment of Juvenile Dermatomyositis in Children Under 18 with Major Organ Involvement or Extensive Ulcerative Skin Disease

This protocol is indicated for newly diagnosed patients under 18 years of age with juvenile dermatomyositis who present with major organ involvement or extensive ulcerative skin disease — features that define a severe disease subset at the outset.

The presence of major organ involvement or extensive ulcerative skin disease in juvenile dermatomyositis is a recognised indicator for escalation of the induction regimen. This sub-population requires a more intensive approach than standard moderate disease management.

Success is measured by improvement in functional muscle assessments, reduction in serum muscle enzyme levels, and decrease in physician-rated global disease activity. Clinically inactive disease is formally defined by satisfying at least three of four established validated criteria.