PERIPHERAL ULCERATIVE KERATITIS AND SEQUENTIAL NECROTIZING SCLERITIS IN A PATIENT WITH GRANULOMATOSIS WITH POLIANGIITIS.

Published 2022 - 26th ESCRS Winter Meeting

Reference: P205 | Type: Abstracts | DOI: 10.82333/xb0n-p768

Authors: Javier Vicente Andreu* ¹ , Rosita Lucia Wakfie Corieh ¹ , Jose Javier San Roman Llorens ¹ , Claudia Azpitarte Sanchez-Muros ¹ , Ester Carreno Salas ¹

¹Oftalmología,Fundación Jiménez Díaz,Madrid,Spain

Purpose

To report a case of a patient with Orbital Vasculitis (OV) in the setting of Granulomatosis with Poliangiitis (GPA) in which a Peripheral Ulcerative Keratitis (PUK) and sequential Necrotizing Scleritis (NS) were observed.

Setting

An 80-year-old female diagnosed with GPA with cutaneous, upper and lower respiratory tract and kidney involvement initially treated with Azathioprine. In the course of the disease, she presented with a right orbital B-cell lymphoma and was treated with Rituximab and orbital radiotherapy, with partial response. The patient declined second-line therapy afterwards and she was controlled with magnetic resonance imaging (MRI) showing stability. Patient lost follow-up and came back to consultation ten months later with a painful right eye.

Methods

Best corrected visual acuity (BCVA), slit lamp examination (SLE), intraocular pressure (IOP), dilated fundus examination (DFE), and magnetic resonance imaging (MRI).

Results

At presentation BCVA was counting fingers on the right eye and IOP was normal. The patient had exotropia and all ocular movements were impaired. SLE showed a superonasal crescent-shaped PUK with marked corneal thinning. The adjacent sclera was hyperemic without nodules or thinning. There were no remarkable DFE findings and MRI showed stability of orbital lesions. Intravenous Methylprednisolone (IVMP) was administered for 3 days. Posteriorly one intravenous Cyclophosphamide pulse was given. There was a good response with improvement of BCVA (20/70) and healing of the PUK area and she was discharged with oral Cyclophosphamide and Prednisone in a tapering regime. She did not attend further follow-up and came back six weeks later with complete healing of PUK but new onset nodular scleritis. IVMP was again administered with good response. The patient lost follow-up again and came three months later to the emergency department with a painful right eye. SLE revealed a NS with marked scleral thinning in the same location of prior nodular scleritis. Neither decrease in VA nor posterior scleral involvement were found. IVMP was given with significant improvement and oral Cyclophosphamide was re-started.

Conclusion

GPA is a severe multisystemic autoimmune disease that is defined by granulomatous inflammation and vasculitis in small and medium-sized vessels. Ocular and orbital manifestations are common in these patients and can potentially involve every structure with a wide range of severity. High-dose immunosuppressors are often needed during flare-ups and multidisciplinary management is key to ocular function preservation. OV, PUK and NS are examples of severe ocular manifestations of GPA that happened nearly simultaneously in our patient and were successfully treated with high-dose corticosteroids and Cyclophosphamyde despite low adherence to treatment and follow-up.