Abstract
Purpose: :
To report two cases of Idiopathic Panuveitis and Retinitis Pigmentosa (RP)
Methods: :
Case 1. A 34-year-old man with nyctalopia and blurry vision in both eyes (OU) was referred due to recalcitrant idiopathic panuveitis and macular edema. He had a positive family history of RP. He had been treated with oral methotrexate (MTX), chlorambucil, mycophenolate mofetil, cyclophosphamide and cyclosporine. Visual acuity (VA) was 20/50 in the right eye (OD) and 20/60 in the left eye (OS). He had anterior chamber and vitreous inflammation, posterior subcapsular cataracts OU, perivascular retinal pigment epithelium changes OU, and papilledema OU. Electroretinogram (ERG) disclosed decrease in amplitude and delayed implicit time OU. Case 2. A 74-year-old female was referred due to persistent blurry vision OS. She was diagnosed with atypical retinitis pigmentosa thirty years earlier. She subsequently developed multifocal choroiditis and panuveitis (MCP), OS and was started on daily mycophenolate mofetil and vitamin A palmitate. VA was no light perception OD and 20/30 OS. She had a 4+ nuclear sclerosis OD and posterior chamber intraocular lens (PCIOL) OS. Fundus exam showed vitreous inflammation, attenuated vessels, granular macula, and diffuse atrophy in the periphery with punched out white lesions in the midperiphery OS.
Results: :
Case 1. Pulsed intravenous MTX improved his VA to 20/40 OD and 20/50 OS, and controlled the panuveitis. Cataract extraction and PCIOL implantation improved VA to 20/30 OD and 20/40 OS. Case 2. Mycophenolate mofetil and vitamin A were restarted. The inflammation was put into remission with this therapy, and the patient has preserved her vision.
Conclusions: :
To our knowledge, these are the first cases in which co-existent panuveitis and RP are reported. In patients with refractory panuveitis presenting with progressive decline in vision and visual field constriction, masquerade syndromes such as retinitis pigmentosa, must be excluded. However, in the event that uveitis masquerade is excluded, the possibility of co-existent RP and genuine uveitis must be considered, in which case immunomodulatory therapy may be used successfully in these patients, with preservation of vision which would otherwise have been lost secondary to inflammatory damage.
Keywords: uveitis-clinical/animal model • autoimmune disease • retinal degenerations: hereditary