Severe reactive ischemic posterior segment inflammation in Acanthamoeba keratitis: Case report of a patient with Sjögren’s syndrome

PURPOSE: We report on a case of Acanthamoeba keratitis (AK)-related reactive ischemic posterior segment inflammation following intraocular surgery in a patient with primary Sjögren’s syndrome (PSS). CASE REPORT: A 48-year-old female patient with severe protracted AK underwent uneventful cataract surgery upon development of a corneal scar. Four weeks postoperatively, she experienced a rapid loss of vision to no light perception. Central retinal artery occlusion and ischemic optic neuropathy could be excluded, and a diagnosis of PSS was made. The condition remained unresponsive to systemic steroid treatment and ultimately led to enucleation of the globe. Histologic work-up revealed ischemic posterior segment inflammation and Acanthamoeba cysts in the corneal stroma. CONCLUSION: Autoimmune disease may be a risk factor for AK-related severe reactive ischemic posterior segment inflammation, and intraocular surgery can be a trigger to its manifestation.