Affects children younger than 18 years of age.
A chronic disease similar to the adult equivalent and primarily affects skin and muscles.
Associated with significant morbidity and mortality.
Characterized by proximal muscle weakness, calcinosis cutis, cutaneous vasculitis, ulcerations, and vasculopathy affecting the gastrointestinal tract.
Histologic findings of muscle biopsy include perifascicular and perivascular inflammatory infiltrates, muscle degeneration and regeneration and type I and type II fiber atrophy.
Treatment is anecdotal because few randomized trials exist.
Clinical consensus is corticosteroids represent first line of treatment.
In steroid resistant or steroid dependent patients an immunosuppressive drugs is added as a steroid sparing agent.
The two most common immunosuppressive drugs used aremethotrexate and cyclosporine.
Combining immunosuppressive agents and steroids could result in better outcomes, although data demonstrating search benefits is not available.
In a randomized study of prednisone versus prednisones plus cyclosporine, versus prednisone plus methotrexate the combination of agents was more effective than steroids alone (Ruperto N et al).