Studija slučaja
Safety and Efficacy of Long-term Use of Infliximab in Severe Juvenile Dermatomyositis – 12 Years of Follow-up
Nastasia Kifer
; Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Marijan Frković
; Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Sanja Perić
; Department of Ophthalmology, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Mario Šestan
; Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Nenad Vukojević
; Department of Ophthalmology, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Marija Jelušić
; Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Sažetak
Juvenile dermatomyositis with emphasized vascu-
lopathy is rare, but the most severe form of the disease, with a
poor prognosis with relapsing and chronic course or, in some cas-
es, 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 hem-
orrhages, 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 grad-
ual 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.
Ključne riječi
juvenile dermatomyositis; TNF inhibitor; infliximab; retinal vasculopathy; treatment efficacy
Hrčak ID:
326854
URI
Datum izdavanja:
10.4.2024.
Posjeta: 529 *