Abstract
Purpose :
To describe various retinal manifestations of a rare, complement-driven disease known as membranoproliferative glomerulonephritis.
Methods :
Retrospective single-center observational case series of patients with ocular pathology and renal biopsy-confirmed membranoproliferative glomerulonephritis, seen between 2006-2023. Multimodal imaging and electronic health records were reviewed for demographic parameters and disease course, including any changes in visual acuity or ocular pathology.
Results :
A total of 6 patients (12 eyes) with a mean age at presentation of 32.8 (SD = 14) years and a mean follow-up of 4.5 years were included in the study. Visual acuity remained unchanged in 67% of the patients, and all patients remained with vision better than 6/12 by the end of follow-up. All patients presented with bilateral drusen at initial visit, primarily in the temporal or macular regions. Fundus autofluorescence revealed these to be hyperfluorescent. Pigmentary changes were observed in 4/12 eyes, with 2/12 eyes having over 12 years of follow up and demonstrable development and progression of retinal pigment epithelium atrophy without foveal involvement. Additionally, optical coherence tomography revealed vertical hyperreflective lesions/outer retinal columnar abnormalities (ORCA), as well as cystoid macular edema in 2 eyes.
Conclusions :
Basal laminar drusen were seen bilaterally in both type I and type II membranoproliferative glomerulonephritis. Yet it is of interest to observe the phenotypic variation in this rare complement-driven disease, with additional findings such as outer retinal columnar abnormalities (ORCA), cystoid macular edema and progressive retinal pigment epithelium atrophy that may be seen in longitudinal follow-up. Future studies genotyping the patient cohort would be of interest to understand any underlying molecular mechanisms.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.