eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Fuchs Heterochromic

Mansoor Arif, MB, BS, Research Officer, Department of Ophthalmology, Aga Khan University Medical College
C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution; Ira G Wong, MD, MS, Associate Director, Clinical Affairs, Uveitis Service, The Francis I Proctor Foundation for Research in Ophthalmology, University of California at San Francisco; Clinical Professor, Department of Ophthalmology, University of California at San Francisco and Stanford University School of Medicine

Updated: Dec 11, 2008

Introduction

Background

First described by Fuchs in 1906, Fuchs heterochromic iridocyclitis (FHI) is a chronic, unilateral iridocyclitis characterized by iris heterochromia.^1,2,3 Fuchs speculated that an unknown process leads to the development of abnormal uveal pigment with chronic low-grade inflammation, eventually causing iris atrophy and secondary glaucoma. Later, he described 38 cases and reported the histopathology of 6 eyes. The uveitis typically occurs in the lighter colored eye of a young adult with minimal ocular symptoms, no pain, and redness of the external eye or meiosis; no related systemic disease is present. Gradual progression of the disease is associated with cataract formation; glaucoma; and, occasionally, vitreous cellular infiltrates. Although typically presenting as a unilateral condition, 7.8-10% of patients have bilateral disease.

Like many syndromes of unknown etiology, the defining characteristics for FHI have expanded over time. Some atypical findings in patients with FHI include absence of heterochromia, reversed heterochromia, and small foci of peripheral choroiditis. FHI is a diagnosis of exclusion. Other forms of infectious and noninfectious uveitis should be suspected and evaluated in patients with unilateral uveitis.

Pathophysiology

The trigger for inflammation of the iris and the ciliary body is unknown. Several unsubstantiated theories have been proposed, including infection from Toxoplasma gondii, an immune dysfunction, infiltration of sensitized lymphocytes, and chronic herpetic infection.^4,5,6,7 Additionally, because iris heterochromia occurs in congenital Horner syndrome, a neurogenic factor contributing to inflammation and structural changes has been proposed.

Iris heterochromia develops as a result of gradual, progressive, irreversible atrophy of the iris stroma. However, some patients with lightly colored irides present with a darkening of the affected eye, because the stromal atrophy allows more visualization of the darkly pigmented iris pigment epithelium posteriorly.⁸

Frequency

United States

FHI is uncommon in the general ophthalmic practice. Because of a lack of symptoms and minimal signs of inflammation, the disease probably is underdiagnosed. In surveys, 2-11% of patients with uveitis have FHI, while 2-17% of patients with anterior uveitis have FHI.^4,9,10,11,12

International

A multicenter study in Spain reports the prevalence of FHI to be 1.3%.

Race

No ethnic or racial predilection exists.

Sex

No sexual predilection exists.

Age

The age at presentation ranges from 20-60 years; the mean age is 40 years.

Clinical

History

The condition may be detected in the asymptomatic patient during a routine eye examination.14

• Low-grade inflammation usually persists over many years and generally does not require treatment.
• In a few patients, inflammation flares up to a moderate level, requiring a short course of topical corticosteroid therapy. High-dose or long-term topical corticosteroids generally are not beneficial to patients with FHI. In fact, long-term therapy will hasten the development of complications, such as cataracts and glaucoma.
• FHI eventually leads to cataract formation and the development of secondary glaucoma.
  • The cataract initially is of the posterior subcapsular type.
  • Secondary glaucoma is multifactorial in origin. Degenerative changes of the trabecular meshwork are the most common cause of secondary glaucoma. Other factors leading to the development of secondary glaucoma include inflammation of the trabecular meshwork, long-term corticosteroid therapy, inhibition of uveoscleral outflow mechanisms, presence of peripheral anterior synechiae, and neovascularization of the trabecular meshwork.

Physical

• The classic triad is heterochromia, cataract, and keratitic precipitates (KPs).15,16
• Conjunctiva and sclera: In most patients, the conjunctiva is uninflamed with no ciliary flush or conjunctival hyperemia; however, prominent conjunctival, episcleral, and scleral vessels may be observed.
• Cornea: Small, nonpigmented, translucent, star-shaped KPs are numerous and are nearly pathognomonic; they have fine filament projections, and, unlike the KPs seen in most patients with uveitis, those in FHI are distributed over the entire posterior corneal surface. Stellate KPs can also be seen in uveitis associated with toxoplasmosis, herpes simplex, herpes zoster, and cytomegalovirus (CMV).
• Anterior chamber: There is minimal anterior chamber cells and flare. Paracentesis may result in the appearance of a filiform hemorrhage (Amsler sign). Amsler sign is not pathognomonic and can occur in other uveitides.
• Iris  
  • Heterochromia is present in most patients. Inflammation is often present in the lighter colored iris; however, reversed heterochromia may be seen, with inflammation present in the eye with the darker colored iris. Initially, heterochromia may not be detectable in patients with a thick iris. Flattening of the anterior iris architecture is seen, resulting from loss of the anterior iris border layer and iris stroma, especially in the peripupillary area. Heterochromia is present in 75-90% cases. It is difficult to see in bilateral cases (5-10%). In unilateral cases, the hypopigmented eye is usually the affected eye. It is difficult to see in very pale blue or very dark brown irides and is no longer essential for diagnosis. It is best detected in daylight or bright overhead light, not at the slit lamp.
  • Iris sphincter atrophy may cause an irregular shaped pupil. Patchy atrophy occurs in the posterior pigment epithelial layer of the iris. There is iris stromal smoothing with loss of the normal corrugated texture.
  • Normally, a lighter colored eye becomes darker when extensive loss of iris stroma occurs, exposing the darker pigment epithelial layer.
  • White iris nodules may be seen along the pupillary border (Koeppe nodules) and in the iris stroma (Busacca nodules).
  • Posterior synechiae are never present. The presence of posterior synechiae should strongly suggest another diagnosis.
  • Iris atrophy and, possibly, iris ischemia lead to fine rubeoticlike vessels on the iris surface, especially in the angle. Fluorescein angiography of the iris demonstrates early leakage and, in some cases, vascular occlusion. Transillumination defects are also present in some cases.
  • Neovascularization of the iris and the anterior chamber angle (radial and circumferential) occurs in 6-22% of cases.
• Trabecular meshwork: Fine blood vessels on the trabecular meshwork may bleed unexpectedly when the intraocular pressure suddenly drops during surgery or paracentesis (Amsler sign).
• Lens: In a 9-year follow-up period, 80-90% of cases developed a posterior subcapsular cataract, which matured rapidly.
• Vitreous: Whitish vitreous cellular infiltrates, varying from dustlike to stringy veils are observed. They are not inflammatory snowballs as seen in intermediate uveitis. They may interfere with sight and require vitrectomy.
• Retina: The absence of cystoid macular edema distinguishes FHI from other uveitis syndromes with chronic vitritis. Chorioretinal scars have been reported in some patients. Cases of retinal breaks and retinal dialysis have been reported.17 A causal relationship has not been identified, but the incidence of retinal dialysis in FHI was reported as almost twice that of the general population. This suggests that a thorough retinal examination is essential in patients suspected of having FHI.
• Intraocular pressure: Secondary glaucoma is a frequent complication and can be present in 15-59% of cases.

Causes

The cause of FHI is not known; however, there are a variety of theories.

• The sympathetic/neurogenic theory supports the idea of adrenergic dysfunction leading to iris hypopigmentation by reduced innervation to iris stromal melanocytes. Abnormal innervation to iris vasculature may account for the breakdown in the blood-aqueous barrier observed with secondary leakage of proteins, cells, and inflammatory mediators into the anterior chamber. This can be supported by the fact that electron microscopy of iridectomy specimens from patients with FHI showed changes in myelinated nerves and associated changes in melanosomes.
• Infectious causes leading to FHI have also been extensively studied. Both FHI and ocular toxoplasmosis have well-defined clinical features, and unequivocal diagnoses can often be made. A strong association between FHI and ocular toxoplasmosis has been documented. T gondii has been suggested as a possible etiologic agent. In Brazil and France, 60% of patients with FHI are reported to have chorioretinal lesions consistent with toxoplasmosis.⁴
  • Toledo de Abreu and coworkers reported chorioretinal scars characteristic of ocular toxoplasmosis in 13 of 23 patients with FHI. All of these patients had characteristic features of both toxoplasmosis and FHI, with positive serum immunofluorescent reactions for toxoplasmosis.¹⁸
  • In 25 patients studied by Schwab, 16 patients had fundus lesions suggestive of ocular toxoplasmosis; 13 of these patients had a positive serologic test for toxoplasmosis, suggesting a significant association between FHI and the chorioretinal scars of toxoplasmosis.⁴
  • Two cases of FHI in otherwise healthy men, aged 26 years and 29 years, respectively, have been described by de Groot-Mijnes and associates. These patients tested positive for the antirubella antibody from aqueous humor isolates. However, no nucleic acid was isolated for any known pathogen from the anterior chamber of these patients.19
• Quentin and Reiber compared aqueous humor samples from patients with FHI with those from patients with other acute and chronic ocular inflammatory conditions and cataract control subjects to determine antibody reactivity against various organisms (eg, rubella virus, varicella zoster virus, herpes simplex virus [HSV], Toxoplasma infection).20,21
  • They calculated an antibody index for each antigen to determine the fraction of specific antibody to total immunoglobulin G (IgG). They found an increased antibody index against rubella in all 52 patients with FHI compared with cataract control subjects and patients with other uveitides. These findings have been supported by de Groot-Mijnes and associates.
  • There has been an isolated case report regarding the detection of HSV DNA by polymerase chain reaction (PCR) in the aqueous humor of a patient with FHI and a possible role for HSV in the pathogenesis.
  • A case of a 24-year-old woman relating FHI with unilateral ocular Toxocara canis has been reported by Teyssot and associates. The isolated findings of HSV and T canis in association with FHI are likely to be insignificant.²²
• There have been 12 case reports of FHI being associated with retinitis pigmentosa, 2 of them with bilateral FHI. However, no significant positive human leukocyte antigen (HLA) associations have been found.
• Immunologic theories attempting to explain the cause of FHI have been put forward. Elevated aqueous interleukin 6 (IL-6) levels have been found in patients with Fuchs uveitis syndrome (FUS) when compared with control subjects.24
• Using ELISA, elevated levels of IgG subclass 1 in the aqueous have been reported when compared to that in patients with other uveitides and cataract control subjects. An antigenic stimulus may lead to local immune dysfunction with IgG subclass 1 production, which may play a role in the pathogenesis.
• A suggestive association of FHI with cytotoxic T cell antigen 4 gene polymorphism has been described by Spriewald and associates. This may potentially be a candidate gene for FHI.²⁵

Differential Diagnoses

Other Problems to Be Considered

Hemosiderosis
Ocular ischemia
Pars planitis
Heterochromia without inflammation
Iris nevus syndrome

Workup

Laboratory Studies

• No laboratory studies are useful to the clinician in making the specific diagnosis of Fuchs heterochromic iridocyclitis (FHI). The diagnosis is based on both the clinical history and the physical examination.
• When the presentation is not typical of FHI, selected laboratory evaluation may be useful to rule out other forms of uveitis that share some clinical characteristics.
  • Angiotensin-converting enzyme (ACE) may be useful to the clinician in diagnosing sarcoid uveitis.
  • Microhemagglutinin test for Treponema pallidum aids in the diagnosis of syphilis.
  • Purified protein derivative (PPD) is beneficial to the clinician in diagnosing tuberculosis.

Imaging Studies

• Imaging studies are not useful in the evaluation of patients with Fuchs heterochromic uveitis.
• Chest x-ray may be beneficial in helping to diagnose those patients with sarcoid uveitis.

Other Tests

• Fluorescein angiography and optical coherence tomography are used in patients with cystoid macular edema.

Procedures

• Previously, anterior chamber paracentesis was considered a diagnostic test for FHI. This procedure is no longer indicated for this purpose.

Histologic Findings

The loss of pigment from the anterior stroma with hyalinization of blood vessel walls and cellular infiltration was described by Fuchs in 1906. Pathological studies show a combination of inflammatory, degenerative, and atrophic changes. The iris and the ciliary body have a low-grade chronic inflammatory cell infiltration of lymphocytes and plasma cells. Although lymphocytes are the predominant infiltrating cells, plasma cells, eosinophils, mast cells, and Russell bodies have all been described. Russell bodies may correlate clinically with the appearance of minute, globular iris crystals, which are typical of FUS. The iris and the ciliary body are atrophic with fibrosis and obliteration of the vascular endothelium with a reduced number of melanocytes. Degenerative changes have been observed in the inner wall of the Schlemm canal and in nerve fibers. Electron microscopy of iridectomy specimens from FHI has shown abnormal melanocytes with loss of dendritic processes and damaged myelinated nerve fibers.

Treatment

Medical Care

In general, treatment is not necessary for patients with the typical low-grade inflammation. Symptomatic flare-ups may require short-term topical corticosteroids; however, long-term therapy is not indicated. Unlike other uveitides, topical steroids should not be used to eliminate cells from the anterior chamber as part of the cells and flare is contributed by the breakdown of the blood-aqueous barrier and leakage of inflammatory infiltrate.

Surgical Care

Surgical decisions are related to the development of cataracts and glaucoma in patients with Fuchs heterochromic iridocyclitis (FHI).¹⁴

• Cataracts associated with Fuchs heterochromic uveitis26
  • Overall, the surgical outcome of patients with FHI is equivalent to patients with age-related cataracts. Patients with FHI tend to have better outcomes following cataract extraction than patients with other forms of uveitis.
  • A small group of patients with FHI are at a higher risk for complications. Risk factors for complications include the following:
    • Rubeosis irides that lead to hyphema
    • Glaucoma may be more difficult to control following surgery.
    • Severe iris atrophy with transillumination defects tends to have worse postoperative inflammation.
    • Dilation of the pupil may be difficult.
    • Phacoemulsification tends to have a better outcome in terms of complications when compared to cataracts removed by extracapsular cataract extraction.
  • In planning for cataract extraction in patients with FHI, consider the following:
    • Small incision surgery is recommended to reduce surgical trauma.
    • Clear corneal incision is preferred to avoid blood vessels in the anterior chamber angle.
    • Slow decompression of intraocular pressure is indicated to reduce the risk of hemorrhage from abnormal iris blood vessels.
    • Peripheral iridectomy is not indicated.
    • An acrylic intraocular lens implant is preferred over a silicone lens to decrease the amount of pigment adhering to the lens postoperatively and to prevent uveitis relapse. If possible, the lens should be placed within the capsular bag.
    • Topical prednisolone acetate 1%, 4 times per day, for several days before and after surgery may blunt the inflammation associated with procedure.
• Glaucoma associated with Fuchs heterochromic iridocyclitis  
  • The incidence of glaucoma ranges from 15-59%. Fortunately, most secondary glaucoma associated with FHI can be controlled with antiglaucoma medications. In the severe case, where 2 or 3 types of antiglaucoma medications cannot achieve control, surgical treatment should be considered.
  • In planning glaucoma surgery in patients with FHI, consider the following:
    • Argon laser trabeculoplasty does not appear effective in improving the outflow where trabecular sclerosis and peripheral anterior synechiae are present.
    • Glaucoma filtering procedures in patients with FHI are less successful compared with that for patients with primary open-angle glaucoma. The intraocular inflammation may lead to bleb failure; therefore, strict control of the inflammation with topical steroids during the perioperative period may improve the surgical outcome. Use of antimetabolites also may improve surgical results.
    • Glaucoma drainage implants may improve the outcome of glaucoma surgery for patients with uveitic glaucoma. 
• Management of symptomatic vitreous opacities: Vitrectomy is advisable for visually significant vitreous infiltrates. It successfully eliminates symptoms of floaters and is associated with a better visual outcome when compared to vitrectomy in other uveitides.

Medication

For the infrequent episodes when inflammation increases to a moderate level, the use of a topical ophthalmic corticosteroid solution or suspension for a short period of time is warranted. Cycloplegics are not generally required.

Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisolone acetate suspension 1% (Pred Forte)

Criterion standard with a fine 1-3 µm suspension particle size. Generic preparations may be significantly less potent because of markedly larger particle sizes, approximately 10 µm in diameter. The larger particles decrease effective exposure of drug to ocular surfaces, reducing absorption and potency. Rebounds may occur when switching from proprietary to generic prednisolone acetate 1% suspension on a dose-for-dose basis.
Initial dosage is titrated according to the degree of inflammation. Therapeutic goal is to reduce the inflammation to the level prior to the flare-up.
Drops should be spaced at least 5 min apart to avoid dilution effects. Gentle eyelid closure or punctal occlusion can significantly decrease upper airway irritation and systemic absorption. A second drop given immediately after the initial drop does not increase potency because the conjunctival cul-de-sac only holds about 10 microliters, and each drop contains 40 microliters.

Dosing

Adult

1 gtt q2h day 1, then taper frequency over following days based on clinical response

Pediatric

Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms also may exist, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.

Topical formulations provide reasonable anti-inflammatory therapeutic effect without the major adverse effects of steroids. Although very potent analgesics, NSAID drops are generally far less potent than steroids.

Diclofenac (Voltaren)

Inhibits prostaglandin synthesis by decreasing activity of enzyme cyclooxygenase, which, in turn, decreases formation of prostaglandin precursors. May facilitate outflow of aqueous humor and decrease vascular permeability.

Dosing

Adult

1 gtt into affected eye qid; continue for a maximum of 2 wk

Pediatric

Not established

Interactions

Additive effect with systemic NSAIDs may occur

Contraindications

Documented hypersensitivity; avoid during pregnancy

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Corneal thinning may occur

Ketorolac (Acular)

Inhibits prostaglandin synthesis by decreasing activity of the enzyme cyclooxygenase, resulting in decreased formation of prostaglandin precursors, which, in turn, results in reduced inflammation.

Dosing

Adult

1 gtt into affected eye qid for up to 2 wk

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Perform ophthalmologic studies in patients who develop eye complaints during therapy; discontinue therapy if changes are noted; changes may include blurred or diminished vision, corneal deposits and retinal disturbances, scotomata, changes in color vision, and macula degeneration

Follow-up

Further Outpatient Care

• Patients should receive follow-up care as needed.

Complications

• Cataracts
• Secondary glaucoma
• Vitreous debris that obscures vision
• Dilated pupil
• Corneal edema (rare)
• Retinal detachment (rare)

Prognosis

• The low-grade inflammation smolders over decades. Initially, vision is not significantly impaired.
• All patients eventually develop cataracts that require surgical removal.
  • Generally, the outcome of cataract surgery with posterior chamber intraocular lens implantation is good. Some patients develop increased inflammation and transient increased intraocular pressure during the postoperative period.
  • Particular care should be given to placement of the intraocular lens within the capsular bag and the use of minimally adherent intraocular lens materials most compatible with uveitis. Acrylic intraocular lenses and surface modified polymethylmethacrylate (PMMA) intraocular lenses appear to be the most desirable materials, while first-generation silicone may create more cellular adhesion and, thus, more inflammation postoperatively.
• Secondary glaucoma is not uncommon. This may require surgical therapy if antiglaucoma medications do not control the disease. The success for glaucoma surgery is less than that for primary open-angle glaucoma.
• Severe vitreous inflammatory debris not only obscures vision but also may create a capacitance for inflammatory mediators. Thus, elective pars plana vitrectomy may not only improve vision but also reduce long-term inflammatory damage to the posterior segment. Vitrectomy may be performed concomitantly with cataract extraction and intraocular lens implantation or as a subsequent procedure.

References

1. Jones NP. Fuchs' heterochromic uveitis: an update. Surv Ophthalmol. Jan-Feb 1993;37(4):253-72. [Medline].

2. Jones NP. Fuchs Heterochromic Uveitis: A reappraisal of the clinical spectrum. Eye. 1991;5 (Pt 6):649-61. [Medline].

3. Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. I. Clinical characteristics of the syndrome. Surv Ophthalmol. May-Jun 1973;17(6):394-457. [Medline].

4. Schwab IR. The epidemiologic association of Fuchs' heterochromic iridocyclitis and ocular toxoplasmosis. Am J Ophthalmol. Mar 15 1991;111(3):356-62. [Medline].

5. Chee SP, Jap A. Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. Dec 2008;146(6):883-9.e1. [Medline].

6. Jurkunas UV, Bitar MS, Rawe IM. Co-localization of Increased Transforming Growth Factor Beta Induced Protein (TGFBIp) and Clusterin Expression in Guttae of Fuchs Endothelial Corneal Dystrophy Patients. Invest Ophthalmol Vis Sci. Nov 14 2008;[Medline].

7. Van Gelder RN. Idiopathic no more: clues to the pathogenesis of Fuchs heterochromic iridocyclitis and glaucomatocyclitic crisis. Am J Ophthalmol. May 2008;145(5):769-71. [Medline].

8. Melamed S, Lahav M, Sandbank U, et al. Fuch's heterochromic iridocyclitis: an electron microscopic study of the iris. Invest Ophthalmol Vis Sci. Dec 1978;17(12):1193-9. [Medline].

9. Wakefield D, Chang JH. Epidemiology of uveitis. Int Ophthalmol Clin. 2005;45(2):1-13. [Medline].

10. Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol. May 1996;114(5):593-9. [Medline].

11. Gritz DC, Wong IG. Incidence and prevalence of uveitis in Northern California, the Northern California Epidemiology of Uveitis Study. Ophthalmology. 2004;111:491-500.

12. Tran VT, Auer C, Guex-Crosier Y, et al. Epidemiological characteristics of uveitis in Switzerland. Int Ophthalmol. 1994-1995;18(5):293-8. [Medline].

13. Norrsell K, Sjodell L. Fuchs' heterochromic uveitis: a longitudinal clinical study. Acta Ophthalmol. Feb 2008;86(1):58-64. [Medline].

14. Mohamed Q, Zamir E. Update on Fuchs' uveitis syndrome. Curr Opin Ophthalmol. Dec 2005;16(6):356-63. [Medline].

15. Liesgang TJ. Fuchs uveitis syndrome. In: Pepose JS, Holland GS, Wilhelmus KR, eds. Ocular Infection & Immunity. 1995:495-506.

16. Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. II. Etiology and mechanisms. Surv Ophthalmol. 1973;18:2-61.

17. Sungur G, Hazirolan D, Duman S, et al. Fuchs' heterochromic uveitis associated with retinal break or dialysis. Can J Ophthalmol. Feb 2008;43(1):109-10. [Medline].

18. Toledo de Abreu M, Belfort R Jr, Hirata PS. Fuchs' heterochromic cyclitis and ocular toxoplasmosis. Am J Ophthalmol. Jun 1982;93(6):739-44. [Medline].

19. de Groot-Mijnes JD, ten Dam-van Loon NH, Weersink AJ, et al. [Relationship between rubella virus and Fuchs heterochromic uveitis; 2 patients]. Ned Tijdschr Geneeskd. Nov 24 2007;151(47):2631-4. [Medline].

20. Quentin CD, Reiber H. Fuchs heterochromic cyclitis: rubella virus antibodies and genome in aqueous humor. Am J Ophthalmol. Jul 2004;138(1):46-54. [Medline].

21. de Groot-Mijnes JD, de Visser L, Rothova A, et al. Rubella virus is associated with fuchs heterochromic iridocyclitis. Am J Ophthalmol. Jan 2006;141(1):212-214. [Medline].

22. Teyssot N, Cassoux N, Lehoang P, et al. Fuchs heterochromic cyclitis and ocular toxocariasis. Am J Ophthalmol. May 2005;139(5):915-6. [Medline].

23. Murray PI, Hoekzema R, van Haren MA, et al. Aqueous humor interleukin-6 levels in uveitis. Invest Ophthalmol Vis Sci. May 1990;31(5):917-20. [Medline].

24. Labalette P, Caillau D, Grutzmacher C, et al. Highly focused clonal composition of CD8(+) CD28(neg) T cells in aqueous humor of fuchs heterochromic cyclitis. Exp Eye Res. Sep 2002;75(3):317-25. [Medline].

25. Spriewald BM, Lefter C, Huber I, et al. A suggestive association of fuchs heterochromic cyclitis with cytotoxic T cell antigen 4 gene polymorphism. Ophthalmic Res. 2007;39(2):116-20. [Medline].

26. Mohammadpour M, Jabbarvand M. Simultaneous phacoemulsification and DSEK in patients with concomitant cataract and bullous keratopathy due to Fuchs endothelial dystrophy. J Cataract Refract Surg. Oct 2008;34(10):1615; author reply 1615-6. [Medline].

Keywords

Fuchs heterochromic uveitis, Fuchs' heterochromic uveitis, Fuchs heterochromic iridocyclitis, Fuchs' heterochromic iridocyclitis, FHI, Fuchs' heterochromic cyclitis, Fuchs' uveitis syndrome

Contributor Information and Disclosures

Author

Mansoor Arif, MB, BS, Research Officer, Department of Ophthalmology, Aga Khan University Medical College
Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi
Disclosure: Nothing to disclose.

Ira G Wong, MD, MS, Associate Director, Clinical Affairs, Uveitis Service, The Francis I Proctor Foundation for Research in Ophthalmology, University of California at San Francisco; Clinical Professor, Department of Ophthalmology, University of California at San Francisco and Stanford University School of Medicine
Ira G Wong, MD, MS is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

John D Sheppard Jr, MD, MMSc, Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Program Director, Ophthalmology Residency Training, Eastern Virginia Medical School; President, Virginia Eye Consultants
John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)