Usher syndrome associated with Fuchs’ heterochromic uveitis

Abstract

This study confirms the association between FHU and RP; and a particularly stronger association with Usher syndrome type II. Although infectious agents seem to play a role, the cause for this significant correlation is still unclear.

Usher syndrome associated with Fuchs’ heterochromic uveitis: a case report

“…Its frequency is reported to be 1/20000 1. Usher syndrome is the most common syndrome associated with retinitis pigmentosa,5 and is known to accompany 14% of cases 4. There are many reports of the association of Fuchs’ heterochromic uveitis and retinitis pigmentosa.…”

“…49 The etiology of Fuchs’ heterochromic uveitis is not clear, and definitions involve many concurrent retinal diseases 3–6. The association of retinitis pigmentosa and Fuchs’ heterochromic uveitis was first reported in 1940,4 and assumed to be coincidental. Since then, there have been 14 reported cases of retinitis pigmentosa associated with Fuchs’ heterochromic uveitis, and the association is no longer regarded as a coincidence 4,7.…”

“…The association of retinitis pigmentosa and Fuchs’ heterochromic uveitis was first reported in 1940,4 and assumed to be coincidental. Since then, there have been 14 reported cases of retinitis pigmentosa associated with Fuchs’ heterochromic uveitis, and the association is no longer regarded as a coincidence 4,7. An autoimmune process is believed to facilitate the ocular inflammation in retinitis pigmentosa 7.…”

“…An autoimmune process is believed to facilitate the ocular inflammation in retinitis pigmentosa 7. The association of Usher syndrome and Fuchs’ heterochromic uveitis has only been reported twice in the literature 4. Both were in patients with type 2 Usher syndrome.…”

“…Its reported incidence is 0.2% 3,4. Fuchs’ heterochromic uveitis accounts for 1%–6% of all cases of uveitis 4.…”