Retinitis pigmentosa associated with Fuchs' heterochromic uveitis

Objective: To investigate whether the combination of Fuchs' heterochromic u veitis (FHU) and retinitis pigmentosa (RP) in the same patient is coinciden tal or represents a true association. Methods: We have examined the frequency of FHU in 338 patients with RP and in 1984 patients who were seen in our primary care ophthalmic clinic becaus e of reasons other than RP. Results: Of 338 patients with RP, 4 (1.2%) had the typical findings of FHU. Three of them had Usher syndrome type II, and 1 had RP simplex. By contras t, only 1 patient in the control group had FHU (5%), and the difference in the frequency of FHU between the 2 groups was significant (P=.002, Fisher e xact test). Conclusions: Fuchs' heterochromic uveitis is associated with RP. Since auto immune phenomena have been previously described in patients with RP, it is conceivable that RP predisposes to the development of FHU.