Safety and Efficacy of Long-term Use of Infliximab in Severe Juvenile Dermatomyositis - 12 Years of Follow-up.

Abstract

Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery. To the best of our knowledge, this is the first long-term follow-up of an early TNF inhibitor introduction in a patient with acute, severe form of juvenile dermatomyositis and retinal vasculopathy. After 12 years of infliximab therapy, the outcome was excellent, with no side effects throughout the whole treatment.