Noninfectious Uveitis Syndromes

Abstract

Noninfectious uveitis syndromes are a heterogeneous group of disorders affecting the retina and choroid. Their etiology is unknown, but some follow a flu-like illness. Autoimmune pathogenesis has also been postulated. Patients are usually affected from the 2nd to 5th decade, and some syndromes show a female preponderance. The presenting symptoms include photopsias, floaters, decreased visual acuity, and visual field loss. Fundus lesions are bilateral (apart from MEWDS) but may be asymmetric and consist of multiple, discrete, yellow-white, well-circumscribed lesions at the level of the outer retina, RPE, and choroid. Multimodal imaging plays an important role in the diagnosis, as each syndrome exhibits a different pattern. Lesions are often more numerous on imaging than clinically evident. Electrophysiology is within normal limits in some syndromes but can be markedly attenuated in others. Prognosis is widely variable and ranges from a self-limiting course with complete resolution of symptoms to relentless progression of visual loss.

Keywords: AIM; AMN; APMPPE; AZOOR; Acute idiopathic maculopathy; Acute macular neuroretinopathy; Acute posterior multifocal placoid pigment epitheliopathy; Acute zonal occult outer retinopathy; Birdshot chorioretinopathy; MEWDS; Multiple evanescent white dot syndrome; Noninfectious uveitis syndromes; PIC; Phenocopies; Punctate inner choroidopathy; Serpiginous choroiditis.