Objective: To report the diagnosis, radiologic findings, and therapy of a 5
1-year-old female with systemic lupus erythematosus (SLE) who, while on hyd
roxychloroquine maintenance therapy, presented with a junctional scotoma in
dicative of chiasmal disease. This visual loss developed after she had been
tapered off corticosteroids.
Materials and Methods: An interventional case report of a female that was g
iven acute therapy with 1-gram daily of intravenous methylprednisolone sodi
um succinate for 5 days, followed by maintenance methotrexate and a slow ta
per of oral prednisone. Magnetic resonance imaging (MRI) scans. visual acui
ty, color vision, and threshold visual fields were performed.
Results: The MRI scan showed chiasmal involvement, which may occur in SLE i
n absence of any other evidence of systemic activity. Therapy led to visual
function returning to 20/20 OD and 20/20 OS, with normal Ishihara plates O
U and only minimal paracentral depressions OU. She has been able to be wean
ed off prednisone while on methotrexate maintenance.
Conclusions: Chiasmal involvement may occur in SLE in absence of any other
evidence of systemic activity. Maintenance with hydroxychloroquine may not
be adequate to prevent this rare cause of visual loss in SLE. Aggressive th
erapy of chiasmal involvement in SLE, even when the visual loss is profound
, may lead to visual restoration, which was virtually complete in this case
. Methotrexate may be an alternate agent for patients who break through wit
h optic neuropathy while on hydroxychloroquine.