Iris nodules in Fuchs' heterochromic uveitis.

Four black patients with Fuchs' heterochromic uveitis had multiple nodules in the iris. The iris nodules were small, transparent, and scattered across the whole surface of the iris (Busacca's nodules) but increased in density toward the pupillary border. All four patients also had nodules at the pupillary border (Koeppe's nodules). Repeated etiologic screening for systemic diseases in these patients was noncontributory. Unilateral multiple iris nodules without the formation of synechiae may be encountered as part of Fuchs' heterochromic uveitis syndrome and may be important in the identification of Fuchs' heterochromic uveitis, especially in black patients.

Quantitative analysis of iris translucency in Fuchs' heterochromic cyclitis.

PURPOSE: To measure intraocular stray light and to quantify translucency of the iris and the surrounding ocular wall in both eyes of patients with Fuchs' heterochromic cyclitis; to investigate whether differences in paired eyes could be measured in patients without heterochromia or with a minimal degree of iris atrophy. METHODS: Intraocular stray light was measured by means of the direct compensation technique. A modification of this technique was used to quantify translucency of the iris and the surrounding ocular wall. RESULTS: Intraocular stray light proved to be significantly higher in all patients with Fuchs' heterochromic cyclitis (both eyes) than in normal control subjects. Translucency of the iris and the ocular wall around it were increased in the patients with Fuchs' heterochromic cyclitis, including patients without heterochromia or with a minimal degree of iris atrophy. CONCLUSIONS: Quantitative analysis of translucency was used to determine iris depigmentation in vivo in patients with Fuchs' heterochromic cyclitis. This technique showed that the process of atrophy and depigmentation in patients with Fuchs' heterochromic cyclitis is probably not restricted to the iris, but also occurs in the surrounding ocular wall. More studies, including other uveitis groups, are necessary to investigate if this technique can be used as a diagnostic tool in Fuchs' heterochromic cyclitis.

Treatment and prognosis of secondary glaucoma in Fuchs' heterochromic iridocyclitis.

After reviewing the records of 111 patients with Fuchs' heterochromic iridocyclitis, we studied the therapy and prognosis of secondary glaucoma in 30 of these 111 patients (27%) who had glaucoma or could be considered glaucoma suspects. Maximal medical therapy was unsuccessful in 22 of the 30 patients (73%). Surgical intervention (mostly trabeculectomies, half with 5-fluorouracil) successfully controlled intraocular pressure (< or = 21 mm Hg with or without medication) in 13 of the 18 operated-on patients (72%) after a mean follow-up of 26 months. All successfully operated-on patients retained a visual acuity of 20/80 or better. We had favorable results, possibly because of modern surgical techniques (use of 5-fluorouracil, sodium hyaluronate) or earlier surgical intervention, or both.

Does autoimmunity to S-antigen play a role in Fuchs' heterochromic cyclitis?

Autoimmunity directed against retinal or choroidal antigens has been suggested to play a role in the chorioretinal lesions observed in patients with Fuchs' heterochromic cyclitis. This hypothesis was addressed and patients with Fuchs' heterochromic cyclitis were tested for cellular immunity (migration inhibitory factor assay) against human retinal S-antigen. A significantly higher percentage of patients with Fuchs' heterochromic cyclitis had a positive cellular autoimmune response to S-antigen than healthy controls and other patients with anterior uveitis. This finding is remarkable since Fuchs' heterochromic cyclitis is generally classified as an anterior uveitis and patients with Fuchs' heterochromic cyclitis without chorioretinal lesions also had a positive test. In view of these results and a sensitisation against a corneal antigen reported earlier in Fuchs' heterochromic cyclitis, it is suggested that a chronic low grade grade anterior uveitis or chorioretinitis of unknown origin may cause the release of potent autoantigens in these patients.

Fuchs' heterochromic cyclitis and retinal vascular abnormalities in progressive hemifacial atrophy.

We report a case of progressive hemifacial atrophy with a combination of ipsilateral Fuchs' heterochromic cyclitis and retinal vascular abnormalities. Recently, evidence was found for a neurovascular defect in hemifacial atrophy. Our case not only supports the (clinical) association between Fuchs' heterochromic cyclitis and hemifacial atrophy, but the retinal vascular abnormalities found in this patient add further support to the existence of a neurovascular defect. These findings and our short review of the literature point to the hypothesis of a common sympathetic defect, implicated in the aetiology of both Fuchs' heterochromic cyclitis and progressive hemifacial atrophy. It has to be borne in mind, however, that Fuchs' heterochromic cyclitis has been reported in association with other diseases. It seems likely that although Fuchs' heterochromic cyclitis is a single clinical entity, it may have more than one cause.

Fuchs' heterochromic iridocyclitis is not associated with ocular toxoplasmosis.

To analyze the association between Fuchs' heterochromic iridocyclitis (FHI) and toxoplasmosis, we performed ocular examinations and used various specific laboratory tests to establish a role for Toxoplasma gondii in the pathogenesis of FHI. Results were compared with those for other types of uveitis and healthy controls. Of the 88 patients with FHI, nine (10.2%) had toxoplasmosislike scars, but an association could not be proved by the indirect immunofluorescence antibody test or enzyme-linked immunosorbent assay, or by a test for cellular immunity to Toxoplasma antigen. Analysis of aqueous humor samples for Toxoplasma antibodies in patients with FHI also yielded negative results. On the basis of the negative results of these laboratory tests, we concluded that FHI is not associated with ocular toxoplasmosis.

Immune deposits in iris biopsy specimens from patients with Fuchs' heterochromic iridocyclitis.

To investigate whether Fuchs' heterochromic iridocyclitis may be an immune complex vasculitis, we used an immunofluorescence technique to detect immunoglobulins and complement in iris biopsy specimens from nine patients with Fuchs' heterochromic iridocyclitis, 12 patients with other types of uveitis, and nine patients with glaucoma but without uveitis. No specific immune deposits were observed in the irises of the patients with Fuchs' heterochromic iridocyclitis. Immunoglobulin G, IgA, IgM, and complement were detected in patients with Fuchs' heterochromic iridocyclitis and patients with uveitis, and these results differed significantly (P less than .05) from the group without uveitis. The immune deposits were found only in the iris vessel walls. No light-microscopic evidence of an inflammatory vascular process could be detected. Further studies are necessary to investigate whether the immune reactants originate from the circulation or result from local formation.

Clinical analysis of Fuchs' heterochromic cyclitis.

Fuchs' heterochromic cyclitis (FHC) is an important diagnosis to make. Not only for the patient, because incorrect diagnosis may lead to unnecessary therapy and the failure to detect secondary glaucoma, but also for the comparison of studies on the etiology of FHC, which is still unknown. No clinical criteria for establishing the diagnosis of FHC have been internationally accepted yet. By means of clinical analyses of FHC patients in different parts of the world, predominant clinical features may be distinguished and combined to form (internationally accepted) diagnostic criteria. We report a clinical analysis of 51 FHC patients in the Netherlands. Acute symptoms (severe redness, pain or photophobia) were never (100%) encountered. Characteristic keratic precipitates (88%) and/or minimal aqueous cells and flare (60%) and/or vitreous opacities (84%) were major signs, indicating a chronic inflammatory activity, in which no synechiae (100%) were present. Heterochromia (82%) was not a constant sign, but iris stromal atrophy, which causes the heterochromia, was always present (100%). Cataract was present in 82% as a result of the chronic iridocyclitis. Secondary glaucoma was present in 22%. Based on the predominant clinical findings obtained from this analysis of FHC patients, and on data in the literature, we propose clinical diagnostic criteria for FHC. Future studies, also including other uveitis groups, are necessary to confirm these diagnostic criteria.

High incidence of corneal epithelium antibodies in Fuch's heterochromic cyclitis.

Sera obtained from 26 patients with Fuchs' heterochromic cyclitis were examined for the presence of autoantibodies directed against the anterior segment of the eye by means of immunofluorescence techniques. Antibodies against human iris tissue could not be detected, whereas autoantibodies against corneal epithelium were found in almost 90% of cases. This is the first report describing the presence of autoantibodies in patients with Fuchs' heterochromic cyclitis and it provides a further clue that immunological mechanisms might play an important role in the aetiology of Fuchs' heterochromic cyclitis.

The predictive value of serum angiotensin converting enzyme and lysozyme levels in the diagnosis of ocular sarcoidosis.

We determined the serum angiotensin converting enzyme and lysozyme levels in 221 patients with uveitis and in 67 control subjects. Angiotensin converting enzyme and lysozyme levels were found to be age dependent. Of the 221 patients, 12 had sarcoidosis. In patients with uveitis who had an angiotensin converting enzyme level above 50 units/l (mean + 2 S.D.), the sensitivity of the test was 84%, the specificity was 95%, and the predictive value was 47%. In these same patients the sensitivity was 60% for a lysozyme level above 8 mg/l (mean + 2 S.D.), the specificity was 76%, and the predictive value was 12%.