Ophthalmic Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome): A Systematic Review of the Literature

Purpose: To review and summarize the clinical features, presentations, diagnostic modalities, and management of ophthalmic manifestations of eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome). Methods: A systematic PubMed search of all English articles on EGPA with ophthalmic involvement was performed. Emphasis was placed on English-language articles, but any article with an abstract translated into English was also included. Only those cases that satisfied the American Rheumatology criteria (1990) for diagnosis were included. Data examined included epidemiology, pathogenesis, presentations, diagnostic modalities, and management. Results: There was a wide range in ophthalmic manifestations of EGPA. In order of most frequent presentation to least frequent, these include central retinal artery or vein occlusion, ischemic optic neuropathy, conjunctival nodules, orbital myositis, proptosis, dacryoadenitis, retinal vasculitis/infarcts/edema, cranial nerve palsy, and amaurosis. The 46 qualifying cases were divided into the categories of ischemic vasculitis versus idiopathic orbital inflammation due to prognostic significance. Ischemic vasculitis cases tended to be older patients (p = 0.03), unilateral (p = 0.006), require immunosuppressive therapy beyond steroids (p = 0.015), and were less likely to show improvement on therapy (p = 0.0003). Conclusions: Prompt diagnosis of EGPA by the ophthalmologist can decrease patient morbidity and mortality. This requires knowledge of likely ophthalmic EGPA presentations, as well as recommended workups and treatment.