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.

A 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 Fuchs heterochromic uveitis (FHU). However, chest radiography may be beneficial in helping to diagnose 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

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 that 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.^{[3, 32]}

Treatment & Management

Jones NP. Fuchs' heterochromic uveitis: an update. Surv Ophthalmol. 1993 Jan-Feb. 37(4):253-72. [QxMD MEDLINE Link].
Jones NP. Fuchs Heterochromic Uveitis: A reappraisal of the clinical spectrum. Eye. 1991. 5 (Pt 6):649-61. [QxMD MEDLINE Link].
Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. I. Clinical characteristics of the syndrome. Surv Ophthalmol. 1973 May-Jun. 17(6):394-457. [QxMD MEDLINE Link].
Schwab IR. The epidemiologic association of Fuchs' heterochromic iridocyclitis and ocular toxoplasmosis. Am J Ophthalmol. 1991 Mar 15. 111(3):356-62. [QxMD MEDLINE Link].
Chee SP, Jap A. Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol. 2008 Dec. 146(6):883-9.e1. [QxMD MEDLINE Link].
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. 2008 Nov 14. [QxMD MEDLINE Link].
Van Gelder RN. Idiopathic no more: clues to the pathogenesis of Fuchs heterochromic iridocyclitis and glaucomatocyclitic crisis. Am J Ophthalmol. 2008 May. 145(5):769-71. [QxMD MEDLINE Link].
Toledo de Abreu M, Belfort R Jr, Hirata PS. Fuchs' heterochromic cyclitis and ocular toxoplasmosis. Am J Ophthalmol. 1982 Jun. 93(6):739-44. [QxMD MEDLINE Link].
La Hey E, Rothova A, Baarsma GS, de Vries J, van Knapen F, Kijlstra A. Fuchs' heterochromic iridocyclitis is not associated with ocular toxoplasmosis. Arch Ophthalmol. 1992 Jun. 110 (6):806-11. [QxMD MEDLINE Link].
de Visser L, Braakenburg A, Rothova A, de Boer JH. Rubella virus-associated uveitis: clinical manifestations and visual prognosis. Am J Ophthalmol. 2008 Aug. 146(2):292-7. [QxMD MEDLINE Link].
Stunf S, Petrovec M, Žigon N, Hawlina M, Kraut A, de Groot-Mijnes JD, et al. High concordance of intraocular antibody synthesis against the rubella virus and Fuchs heterochromic uveitis syndrome in Slovenia. Mol Vis. 2012. 18:2909-14. [QxMD MEDLINE Link]. [Full Text].
Wensing B, Relvas LM, Caspers LE, et al. Comparison of rubella virus- and herpes virus-associated anterior uveitis clinical manifestations and visual prognosis. Ophthalmology. 2011 Oct. 118(10):1905-10. [QxMD MEDLINE Link].
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. 2007 Nov 24. 151(47):2631-4. [QxMD MEDLINE Link].
de Groot-Mijnes JD, de Visser L, Rothova A, et al. Rubella virus is associated with fuchs heterochromic iridocyclitis. Am J Ophthalmol. 2006 Jan. 141(1):212-214. [QxMD MEDLINE Link].
Quentin CD, Reiber H. Fuchs heterochromic cyclitis: rubella virus antibodies and genome in aqueous humor. Am J Ophthalmol. 2004 Jul. 138(1):46-54. [QxMD MEDLINE Link].
Teyssot N, Cassoux N, Lehoang P, et al. Fuchs heterochromic cyclitis and ocular toxocariasis. Am J Ophthalmol. 2005 May. 139(5):915-6. [QxMD MEDLINE Link].
Murray PI, Hoekzema R, van Haren MA, et al. Aqueous humor interleukin-6 levels in uveitis. Invest Ophthalmol Vis Sci. 1990 May. 31(5):917-20. [QxMD MEDLINE Link].
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. 2002 Sep. 75(3):317-25. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Wakefield D, Chang JH. Epidemiology of uveitis. Int Ophthalmol Clin. 2005. 45(2):1-13. [QxMD MEDLINE Link].
Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol. 1996 May. 114(5):593-9. [QxMD MEDLINE Link].
Gritz DC, Wong IG. Incidence and prevalence of uveitis in Northern California, the Northern California Epidemiology of Uveitis Study. Ophthalmology. 2004. 111:491-500.
Tran VT, Auer C, Guex-Crosier Y, et al. Epidemiological characteristics of uveitis in Switzerland. Int Ophthalmol. 1994-1995. 18(5):293-8. [QxMD MEDLINE Link].
Norrsell K, Sjodell L. Fuchs' heterochromic uveitis: a longitudinal clinical study. Acta Ophthalmol. 2008 Feb. 86(1):58-64. [QxMD MEDLINE Link].
Tabbut BR, Tessler HH, Williams D. Fuchs' heterochromic iridocyclitis in blacks. Arch Ophthalmol. 1988 Dec. 106 (12):1688-90. [QxMD MEDLINE Link]. [Full Text].
Jain IS, Gupta A, Gangwar DN, Dhir SP. Fuchs' heterochromic cyclitis: some observations on clinical picture and on cataract surgery. Ann Ophthalmol. 1983 Jul. 15 (7):640-2. [QxMD MEDLINE Link].
Kimura SJ. Fuchs' syndrome of heterochromic cyclitis in brown-eyed patients. Trans Am Ophthalmol Soc. 1978. 76:76-89. [QxMD MEDLINE Link]. [Full Text].
Melamed S, Lahav M, Sandbank U, et al. Fuch's heterochromic iridocyclitis: an electron microscopic study of the iris. Invest Ophthalmol Vis Sci. 1978 Dec. 17(12):1193-9. [QxMD MEDLINE Link].
Mohamed Q, Zamir E. Update on Fuchs' uveitis syndrome. Curr Opin Ophthalmol. 2005 Dec. 16(6):356-63. [QxMD MEDLINE Link].
Liesegang TJ. Clinical features and prognosis in Fuchs' uveitis syndrome. Arch Ophthalmol. 1982 Oct. 100 (10):1622-6. [QxMD MEDLINE Link].
Amsler M. New clinical aspects of the vegetative eye. Trans Ophthalmol Soc UK. 1948. 68:45-74.
Goldberg MF, Erozan YS, Duke JR, Frost JK. Cytopathologic and histopathologic aspects of Fuchs' heterochromic iridocyclitis. Arch Ophthalmol. 1965 Nov. 74 (5):604-9. [QxMD MEDLINE Link].
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. 2008 Oct. 34(10):1615; author reply 1615-6. [QxMD MEDLINE Link].
La Hey E, de Vries J, Langerhorst CT, Baarsma GS, Kijlstra A. Treatment and prognosis of secondary glaucoma in Fuchs' heterochromic iridocyclitis. Am J Ophthalmol. 1993 Sep 15. 116 (3):327-40. [QxMD MEDLINE Link].
Da Mata A, Burk SE, Netland PA, Baltatzis S, Christen W, Foster CS. Management of uveitic glaucoma with Ahmed glaucoma valve implantation. Ophthalmology. 1999 Nov. 106 (11):2168-72. [QxMD MEDLINE Link].
Liesgang TJ. Fuchs uveitis syndrome. Pepose JS, Holland GS, Wilhelmus KR, eds. Ocular Infection & Immunity. 1995. 495-506.
Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. II. Etiology and mechanisms. Surv Ophthalmol. 1973. 18:2-61.
Babu K, Adiga M, Govekar SR, Kumar BR, Murthy KR. Associations of Fuchs heterochromic iridocyclitis in a South Indian patient population. J Ophthalmic Inflamm Infect. 2013 Jan 15. 3(1):14. [QxMD MEDLINE Link]. [Full Text].
Sungur G, Hazirolan D, Duman S, et al. Fuchs' heterochromic uveitis associated with retinal break or dialysis. Can J Ophthalmol. 2008 Feb. 43(1):109-10. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Manolette R Roque, MD, MBA, FPAO Section Chief, Cataract and Refractive Surgery, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City

Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society, European Society of Cataract and Refractive Surgery, International Ocular Inflammation Society, Philippine Academy of Ophthalmology, Philippine College of Surgeons, Philippine Medical Association, Philippine Ocular Inflammation Society, Philippine Society of Cataract and Refractive Surgery, University of the Philippines Medical Alumni Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department 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, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

R Christopher Walton, MD Adjunct Professor, Department of Ophthalmology, University of Texas Health Science Center at San Antonio

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

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, Association for Research in Vision and Ophthalmology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: 1-800-DOCTORS; AbbVie; Alcon; Aldeyra; Allergan; Alphaeon; ArcScan; Baush+Lomb; Bio-Tissue; Clearside; EyeGate; Hovione; Mededicus; NovaBay; Omeros; Pentavision; Portage; Santen; Science Based Health; Senju; Shire; Sun Pharma; TearLab;TearScience;Topivert<br/>Serve(d) as a speaker or a member of a speakers bureau for: AbbVie; Alcon; Allergan; Bausch+Lomb; Bio-tissue; EyeGate;Hovione;LayerBio; NovaBay;Omeros;Portage; Santen; Shire; Stemnion; Sun Pharma;TearLab;TearScience; Topivert <br/>Received research grant from: Alcon; Aldeyra; allergan; Baush+ Lomb; EyeGate; Hovione; Kala; Ocular Therapeutix;Pfizer; RPS; Santen;Senju;Shire;Topcon; Xoma.

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO 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, FARVO 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, Sigma Xi, The Scientific Research Honor Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA); Allakos (Redwood City, CA)<br/>Serve(d) as a speaker or a member of a speakers bureau for: Alcon (Geneva, Switzerland); Allergan (Dublin, Ireland); Mallinckrodt (Staines-upon-Thames, United Kingdom)<br/>Received research grant from: Alcon; Aldeyra Therapeutics; Allakos Pharmaceuticals; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen; Aciont.

Ira G Wong, MD, MS Clinical Professor, Departments of Ophthalmology, University of California at San Francisco and Stanford University School of Medicine; The Francis I Proctor Foundation for Research in Ophthalmology, University of California at San Francisco

Ira G Wong, MD, MS is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Mansoor Arif, MD Hospitalist, Department of Medicine, Mount Auburn Hospital; Instructor, Department of Medicine, Harvard Medical School

Mansoor Arif, MD is a member of the following medical societies: American College of Physicians, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Neerav Neel Lamba, MD, MBA Fellow in Ocular Immunology, Massachusetts Eye Research and Surgery Institution

Neerav Neel Lamba, MD, MBA is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

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