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.

Keywords: Granulomatosis with polyangiitis; Peripheral ulcerative keratitis; Scleritis; Wegener’s granulomatosis.

Fig. 1.

Presenting anterior segment photo of right eye demonstrating nodular scleritis (a) with magnified appearance under slit-lamp (b).

Fig. 2.

Multi-system CT. a Demonstrates extensive polypoid mucosal thickening of the maxillary antrum (m), nasal turbinates (*), ethmoidal and frontal sinuses. b Demonstrates multiple solid lung nodules within the upper (arrow) and lower lobes. c Shows a large bladder mass (b).

Fig. 3.

H&E stained right sclera histopathology sample demonstrating inflamed granulation tissue with reactive atypical squamous epithelium and multinucleate giant cells (*).

Fig. 4.

Progress anterior segment photo 5 months following presentation (a) including with fluorescein sodium stain (b).