eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Fuchs Heterochromic: Differential Diagnoses & Workup

Author: Mansoor Arif, MB, BS, Research Officer, Department of Ophthalmology, Aga Khan University Medical College
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; 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
Contributor Information and Disclosures

Updated: Dec 11, 2008

Differential Diagnoses

Glaucoma, Pigmentary
Posner-Schlossman Syndrome
Glaucoma, Uveitic
Retinitis, CMV
Herpes Simplex
Sarcoidosis
Herpes Zoster
Toxoplasmosis
HIV
Tuberculosis
HLA-B27 Syndromes
Uveitis, Intermediate
Horner Syndrome
Ocular Manifestations of HIV

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.

More on Uveitis, Fuchs Heterochromic

Overview: Uveitis, Fuchs Heterochromic
Differential Diagnoses & Workup: Uveitis, Fuchs Heterochromic
Treatment & Medication: Uveitis, Fuchs Heterochromic
Follow-up: Uveitis, Fuchs Heterochromic
References
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References

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  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].

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  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].

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  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].

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  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].

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Further Reading

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Keywords

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

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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.

 
 
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