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Bilateral necrotizing scleritis and peripheral ulcerative keratitis associated with granulomatosis with polyangiitis

Poster Details

First Author: S.Stanojlovic SERBIA

Co Author(s):    S. Arandjelovic   T. Kalezic   B. Dacic   S. Djuric     

Abstract Details


To report the clinical course and medical treatment of progressive necrotizing scleritis and peripheral ulcerative keratitis (PUK) occurring in both eyes simultaneously, in a patient with generalized form of granulomatosis with polyangiitis (GPA), after remission of pulmonary and renal manifestations was achieved.


University Eye Hospital, Belgrade


Observational case report.


A 61-year-old man with GPA developed bilateral rapidly progressive necrotizing scleritis and PUK. Acute exacerbation of ocular inflammation occurred during maintenance treatment with oral cyclophosphamide (CYP) and 3 months after the induction regimen with 6 CYP pulsed was given. The area of inflammation involved the entire anterior globe and peripheral cornea of both eyes. More than 90% of the surface area healed within 8 weeks following treatment with 3 pulsed doses of methylprednisolon in addition to oral CYP. Although the inflammation was arrested in both eyes, the advanced long-standing PUK in his right eye led to visual loss.


Severe ocular inflammation associated with a generalized GPA may develop during remission of systemic disease, thus, additional anti-inflammatory and immunosuppressive therapy is required. Introduction of high-dose pulsed methylprednisolone, in addition to maintenance dose prednisone and increased oral CYP, arrested bilateral necrotizing scleritis and PUK in a patient with generalized GPA associated with ophthalmic inflammation refractory to CYP induction regimen. FINANCIAL DISCLOUSRE: NONE

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