Abstract
Purpose :
Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disease characterized by inflammation of small to medium sized blood vessels (vasculitis). The goal of our study was to describe the three causes of GPA-associated optic neuropathy (compressive, inflammatory, or ischemic), analyze their initial and final visual acuities, and to compare our findings to the literature.
Methods :
This was a retrospective chart review of patients who were diagnosed with GPA-associated optic neuropathy and were seen in the Department of Ophthalmology at Mayo Clinic in Rochester, Minnesota. Only patients that met the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology GPA classification criteria (ACR/EULAR) were included.
Results :
A total of ten patients with GPA-associated optic neuropathy were identified. The etiology of the optic neuropathy was compressive in 4 patients, inflammatory in 4, and ischemic in 3 patients. One patient had compressive optic neuropathy initially, then presented with GPA-related optic neuritis years later. Average logMAR visual acuity at optic neuropathy onset was 2.15, 1.50, and 0.67 (Snellen equivalent Hand Motion, 20/600, and 20/100), respectively. At last follow-up, average logMAR visual acuity was 1.31, 1.73, and 1.10 (Snellen equivalent 20/400, 20/1000, and 20/250) for each group.
Conclusions :
Our study showed that compressive and inflammatory were the most common etiologies for GPA-associated optic neuropathies. Visual outcomes were variable, with both compressive and inflammatory optic neuropathies showing improvement in some eyes, but worsening in other eyes. Patients with ischemic optic neuropathy from GPA were either stable or worsened over the course of the disease.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.