Ophthalmic Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

A Systematic Review of the Literature

Sruti S. Akella, Dianne M. Schlachter, Evan H. Black, Anne Barmettler

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)7-16
Number of pages10
JournalOphthalmic plastic and reconstructive surgery
Volume35
Issue number1
DOIs
StatePublished - Jan 1 2019
Externally publishedYes

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Churg-Strauss Syndrome
Granulomatosis with Polyangiitis
Eye Manifestations
Vasculitis
Orbital Myositis
Retinal Vasculitis
Dacryocystitis
Retinal Artery Occlusion
Ischemic Optic Neuropathy
Retinal Vein
Cranial Nerve Diseases
Retinal Vein Occlusion
Exophthalmos
Rheumatology
Blindness
Immunosuppressive Agents
PubMed
Edema
Epidemiology
Language

ASJC Scopus subject areas

  • Surgery
  • Ophthalmology

Cite this

Ophthalmic Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) : A Systematic Review of the Literature. / Akella, Sruti S.; Schlachter, Dianne M.; Black, Evan H.; Barmettler, Anne.

In: Ophthalmic plastic and reconstructive surgery, Vol. 35, No. 1, 01.01.2019, p. 7-16.

Research output: Contribution to journalArticle

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