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Fuchs' heterochromic iridocyclitis: causes, symptoms, diagnosis, treatment
Last reviewed: 04.07.2025
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Fuchs' heterochromic iridocyclitis is a unilateral chronic inactive nongranulomatous anterior uveitis associated with secondary posterior subcapsular cataract and glaucoma in 13-59% of cases.
As a result of intraocular inflammation, atrophy of the iris occurs and heterochromia characteristic of this condition appears.
[ Epidemiology of Fuchs' heterochromic iridocyclitis
Fuchs heterochromic iridocyclitis is considered to be a relatively rare form of anterior uveitis, accounting for 1.2 to 3.2% of all uveitis. In 90% of cases, there is unilateral involvement. The disease occurs equally often in men and women. The disease is usually detected at the age of 20-40 years. In 15% of cases, inflammatory glaucoma is detected already at the time of diagnosis of Fuchs heterochromic iridocyclitis, and in 44% of cases it develops later. The overall incidence of secondary glaucoma in patients suffering from Fuchs heterochromic iridocyclitis is 13-59%, but this figure may be higher in patients with bilateral involvement and in the African American population.
What causes Fuchs' heterochromic iridocyclitis?
It is believed that the increase in intraocular pressure in Fuchs' heterochromic iridocyclitis occurs as a result of a disruption in the outflow of intraocular fluid due to obstruction of the trabecular meshwork by inflammatory cells or the hyaline membrane.
Symptoms of Fuchs' heterochromic iridocyclitis
Fuchs' heterochromic iridocyclitis is asymptomatic, with only a few cases of patients complaining of slight discomfort and blurred vision. No connection with systemic diseases has been identified. Patients often see a doctor due to decreased visual acuity as cataracts progress.
Course of the disease
Anterior uveitis in Fuchs heterochromic iridocyclitis progresses slowly and is asymptomatic. Neovascularization of the iris and anterior chamber angle with mild trauma may result in minor intraocular hemorrhage, but peripheral anterior synechiae or neovascular glaucoma do not develop. The most common complications of the disease are cataracts and glaucoma. Cataract formation was noted in 50% of patients with Fuchs heterochromic iridocyclitis. Cataract extraction usually does not cause complications, and postoperative exacerbation of intraocular inflammation is less common than with other uveitis. Posterior chamber implantation of an intraocular lens is safe. Glaucoma developing in Fuchs heterochromic iridocyclitis resembles primary open-angle glaucoma in its course.
Ophthalmological examination
On external examination, the eye is usually calm, without signs of inflammation. Examination of the anterior segment of the eye usually reveals unilateral inactive nongranulomatous anterior uveitis. Stellate precipitates are scattered throughout the corneal endothelium, which is a characteristic diagnostic sign. The intraocular inflammatory process leads to atrophy of the iris stroma, as a result of which the dark iris will appear lighter. In patients with light irises, as a result of stromal atrophy, the affected eye will appear darker due to exposure of the iris pigment epithelium. Another important diagnostic sign in patients with Fuchs' heterochromic iridocyclitis is neovascularization of the iris or the anterior chamber angle (detected by gonioscopy). Despite the chronic course of intraocular inflammation, patients almost never develop peripheral anterior and posterior synechiae. However, posterior subcapsular cataract is a fairly common complication. Usually, the posterior segment of the eye is not affected, but cases of chorioretinal lesions have been described in patients with Fuchs' heterochromic iridocyclitis.
Differential diagnostics of Fuchs' heterochromic iridocyclitis
It is necessary to carry out differential diagnostics of Fuchs' heterochromic iridocyclitis with Posner-Schlossman syndrome, sarcoidosis, syphilis, herpetic uveitis, and, in case of damage to the posterior segment, with toxoplasmosis.
Laboratory research
There are no laboratory tests that can diagnose Fuchs' heterochromic iridocyclitis. Lymphocytes and plasma cells have been found in the intraocular fluid of patients with this disease. The diagnosis is based on clinical manifestations: distribution of precipitates over the corneal endothelium, inactive anterior uveitis, heterochromia, absence of synechiae, and scanty ocular symptoms.
Treatment of Fuchs' heterochromic iridocyclitis
Despite chronic anterior uveitis, aggressive topical glucocorticoid therapy or systemic immunosuppressive therapy is not recommended due to their low efficacy in Fuchs heterochromic iridocyclitis. The use of topical glucocorticoids may even be contraindicated due to their acceleration of cataract and glaucoma development. Drug treatment of glaucoma is recommended, but surgery is required in 66% of cases. The best surgical procedure for treating patients with Fuchs heterochromic iridocyclitis is unknown. Argon laser trabeculoplasty is ineffective in these patients due to the formation of a hyaline membrane over the trabecular meshwork and should not be used.