Fuchs Heterochromic Uveitis 

  • Author: Mansoor Arif, MD, MBBS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Oct 10, 2011
 

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.

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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]

Rubella virus that is well known for causing German measles has been postulated to be involved in the pathogenesis of Fuchs heterochromic uveitis (FHU). The exact molecular mechanisms still remain a topic for research, but the clinical spectrum of rubella-uveitis resembles FHU in many aspects.[9]

Wensing et al found that FHU can also be difficult to clinically distinguish from herpetic anterior uveitis (AU). Although typical FHU anterior segment signs were associated with vitreitis, FHU was diagnosed solely in patients with AU.[10]

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Epidemiology

Frequency

United States

Fuchs heterochromic iridocyclitis (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, 11, 12, 13, 14]

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.

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Contributor Information and Disclosures
Author

Mansoor Arif, MD, MBBS  Research Associate, Department of Research, Indus Hospital, Pakistan

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: Allergan, Inc Consulting fee Review panel membership

Specialty Editor Board

John D Sheppard Jr, MD, MMSc  Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, 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.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

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.

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.

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.

Additional Contributors

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

References

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  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].

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