Ocular involvement in
Churg-Strauss syndrome is infrequent. We describe the case of a 54-year-old woman with
eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for
Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye.
Central retinal artery occlusion (CRAO) without accompanying
retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of
retinal whitening with a cherry-red spot. Although her
antineutrophil cytoplasmic antibody (
ANCA) status was negative, CRAO was thought to be an ocular manifestation of
Churg-Strauss syndrome, and appropriate treatment was planned. She was treated with high-dose
corticosteroids and
anticoagulant therapy. Her
macular edema improved, but visual recovery was poor. Specific
therapy to alter
inflammation, blood coagulation, and rheology reportedly plays an important role in
ANCA-positive patients with
Churg-Strauss syndrome who develop CRAO. Regardless of
ANCA status, high-dose
corticosteroids should be considered for CRAO in patients with
Churg-Strauss syndrome, as discussed in this case.