Abstract
We report a 16-year-old girl who developed neuromyelitis optica（NMO）associated with systemic
juvenile idiopathic arthritis（s-JIA）． She developed left optic neuritis at the age of 10． Since then she experienced
recurrent attacks of optic neuritis and myelitis． She was diagnosed with NMO at the age of 14， and she was
seropositive for NMO-IgG（antibody for aquaporin 4． She was hospitalized after she suffered joint pain of her
bilateral fingers and left knee． At the same time， she experienced remittent fever． She suffered atypical erythema
on her abdomen and femur． Laboratory examination showed that antinuclear antibodies were positive at l：320．
MRI of her right fingers suggested arthritis． Based on the clinical symptoms and MRI findings， we confirmed
a diagnosis of s-JIA． After admission， she developed macrophage activating syndrome and was treated with
dexamethasone palmitate and cyclosporin A． She experienced a recurrent attack of s-JIA at the age of 16 and 9
months old． She had been treated with tocilizumab． We were unable to find a case report of NMO associated with
s-JIA， and therefbre， we consider this as a rare case．
