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The Atypical Red Eye: A Case of Scleritis with Peripheral Ulcerative Keratitis Secondary to Granulomatosis with Polyangiitis.


ABSTRACT: This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvement. His ocular sequelae included significant peripheral corneal thinning requiring cyanoacrylate gluing, juxtalimbal conjunctival resection, and bandage lens placement. He was treated with systemic methylprednisolone and rituximab achieving remission with ongoing prednisone and methotrexate maintenance therapy. This case demonstrates the importance of recognizing ocular manifestations of GPA as a first presentation of systemic vasculitis.

SUBMITTER: Bonnet LA 

PROVIDER: S-EPMC10077138 | biostudies-literature | 2023 Jan-Dec

REPOSITORIES: biostudies-literature

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The Atypical Red Eye: A Case of Scleritis with Peripheral Ulcerative Keratitis Secondary to Granulomatosis with Polyangiitis.

Bonnet Louis Antoine LA   Lipsky Lior L   Holmes Richard R  

Case reports in ophthalmology 20230101 1


This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvemen  ...[more]

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2018-08-29 | GSE119136 | GEO