Abstract
Purpose :
Calpain 5 (CAPN5) associated autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) is a rare heritable autoimmune uveitis which shows features of progressive inflammation, neovascularization, and fibrosis ultimately resulting in phthisis and blindness. The CAPN5 gene encodes a calcium-dependent protease expressed in photoreceptors. Most literature regarding ADNIV treatment was published before the advent of current intravitreal therapies and surgical techniques. We report our long term experience treating 3 related patients with ADNIV.
Methods :
Methods: This was a case series of 3 patients. 6 eyes of 3 patients were included in the review. The eyes were treated with intensive intravitreal anti-VEGF and corticosteroid therapy including, dexamethasone implant, and fluocinolone implant on a regimen by a single surgeon. Most eyes underwent PPV for epiretinal membranes. A single eye underwent RD repair.
Results :
5/6 eyes did not show stage progression and maintained excellent vision with a mean of 0.27 logMAR (20/37 Snellen). These all developed epiretinal membranes which were surgically dissected. A single eye presented with stage 3 ADNIV, and a vision of 20/200 with previous RD repair.
Conclusions :
The natural history of ADNIV involves onset in the first few decades of life with progression through the 5 stages of neovascularization, fibrosis and ultimately retinal detachments. With early intervention and intensive combination intravitreal anti-VEGF and corticosteroid therapy, it is possible to curb the natural progression of this disease and preserve vision.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.