September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
The management and outcomes of Granulomatosis with Polyangiitis associated peripheral ulcerative keratitis: a report of a novel therapy.
Author Affiliations & Notes
  • khawla abusamra
    ophthalmology , MERSI, Cambridge, Massachusetts, United States
    Ocular Immunology and Uveitis Foundation , Cambridge , Massachusetts, United States
  • Nazanin Ebrahimiadib
    ophthalmology , MERSI, Cambridge, Massachusetts, United States
    Ocular Immunology and Uveitis Foundation , Cambridge , Massachusetts, United States
  • C. Stephen Foster
    ophthalmology , MERSI, Cambridge, Massachusetts, United States
    Ocular Immunology and Uveitis Foundation , Cambridge , Massachusetts, United States
  • Footnotes
    Commercial Relationships   khawla abusamra, None; Nazanin Ebrahimiadib, None; C. Stephen Foster , None
  • Footnotes
    Support  none
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 1448. doi:
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      khawla abusamra, Nazanin Ebrahimiadib, C. Stephen Foster; The management and outcomes of Granulomatosis with Polyangiitis associated peripheral ulcerative keratitis: a report of a novel therapy.. Invest. Ophthalmol. Vis. Sci. 2016;57(12):1448.

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      © 2017 Association for Research in Vision and Ophthalmology.

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Abstract

Purpose : The management of granulomatosis with polyangiitis associated peripheral ulcerative keratitis (PUK) represents a real challenge to all ophthalmologists. In this retrospective study we report the use of a novel regimen of rituximab in the treatment of PUK. In addition we report the effectivity of rituximab or cyclophosphamide therapy in the management of PUK

Methods : The patient database at Massachusetts Eye Research Institute (MERSI) was reviewed to identify all patients with peripheral ulcerative keratitis in the setting of granulomatosis with polyangiitis who were evaluated and treated at MERSI between 2005 and 2015

Results : Forty patients with the diagnosis of granulomatosis with polyangiitis were identified; of which 15 patients had peripheral ulcerative keratitis. Nine patients (72%) did not respond to an initial corticosteroid and anti-metabolite (methotrexate, cellcept, imuran) therapy. Those patients were treated with either rituximab or cyclophosphamide infusions. Our rituximab infusion protocol at MERSI was as follows: A dose of 375mg/m2 was infused every week for 8 consecutive weeks and then monthly for an open ended period, terminated depending on the patients’ response. The regimen for cyclophosphamide is a 750-1000 mg infusion every 2 weeks, extended and stopped according to the patient’s response. Seven of the nine (78%) patients showed an outstanding response and the disease was controlled with either regimen. Two of the nine patients did not respond well and they needed corneal grafting to control impending perforation.

Conclusions : In patients with granulomatosis with polyangiitis associated peripheral ulcerative keratitis refractory to classic anti-metabolite treatment, rituximab or cyclophosphamide can successfully stop the progression of the disease and induce remission

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

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