Skip to main content

Advertisement

SpringerLink
Log in

Clinical manifestation of Fuchs uveitis syndrome in childhood

  • Inflammatory Disorders
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

The aim of this study was to describe clinical signs and complications of Fuchs uveitis syndrome (FUS) with onset in childhood.

Methods

Ophthalmologic findings and complications in patients with FUS becoming manifest before the age of 16 years were analyzed in a retrospective study at a tertiary referral uveitis center. Inclusion criteria were the presence of pathognomonic FUS findings at any time point and exclusion of any systemic immune-mediated or infectious disease.

Results

A total of 23 patients (male = 16, female = 7) with juvenile FUS (unilateral n = 20, bilateral n = 3 patients) were included in the study. Mean ages at uveitis and FUS diagnosis were 12.0 ± 4.2 and 22.7 ± 10.7 years, respectively. In six patients, inflammation was noted at age ≤ 7 years. The following inflammatory signs were observed in a total of 26 eyes: ≤ 1+ anterior chamber cell grade (n = 26), vitreous cells (n = 24), fine keratic precipitates (KPs; n = 23), stellate KPs (n = 11), mutton-fat KPs (n = 23), diffuse (n = 24) or inferior (n = 8) distribution of KPs, Koeppe nodules (n = 10), and iris heterochromia (n = 14). A representative subgroup of patients (n = 5) is shown who presented with non-specific clinical signs in the beginning and in whom typical FUS signs became manifest only at a later stage. Secondary complications such as cataract (n = 19), ocular hypertension (n = 3), or glaucomatous disc damage (n = 2) were found after a mean uveitis duration of 11.6, 19.5, and 20.3 years, respectively.

Conclusion

FUS may begin in early childhood, and the characteristic findings may not be present at onset of disease. The diagnosis is often delayed for years, occasionally with the consequence of overtreatment with anti-inflammatory drugs.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Herbort CP, Khairallah M (2010) Editorial: Fuchs’ uveitis: from Imperial Vienna to global appraisal. Int Ophthalmol 30:449–452. doi:10.1007/s10792-010-9383-y

    Article  PubMed  Google Scholar 

  2. Mohamed Q, Zamir E (2005) Update on Fuchs’ uveitis syndrome. Curr Opin Ophthalmol 16:356–363

    Article  PubMed  Google Scholar 

  3. Jones NP (1993) Fuchs’ heterochromic uveitis: an update. Surv Ophthalmol 37:253–272

    Article  CAS  PubMed  Google Scholar 

  4. Rothova A, La Hey E, Baarsma GS, Breebaart AC (1994) Iris nodules in Fuchs’ heterochromic uveitis. Am J Ophthalmol 118:338–342

    Article  CAS  PubMed  Google Scholar 

  5. Tran VT, Auer C, Guex-Crosier Y et al (1994) Epidemiological characteristics of uveitis in Switzerland. Int Ophthalmol 18:293–298

    Article  PubMed  Google Scholar 

  6. Jakob E, Reuland MS, Mackensen F et al (2009) Uveitis subtypes in a german interdisciplinary uveitis center–analysis of 1916 patients. J Rheumatol 36:127–136. doi:10.3899/jrheum.080102

    PubMed  Google Scholar 

  7. Birnbaum AD, Little DM, Tessler HH, Goldstein DA (2011) Etiologies of chronic anterior uveitis at a tertiary referral center over 35 years. Ocul Immunol Inflamm 19:19–25. doi:10.3109/09273948.2010.519852

    Article  PubMed Central  PubMed  Google Scholar 

  8. Cimino L, Aldigeri R, Salvarani C et al (2010) The causes of uveitis in a referral centre of Northern Italy. Int Ophthalmol 30:521–529. doi:10.1007/s10792-010-9359-y

    Article  CAS  PubMed  Google Scholar 

  9. Soylu M, Ozdemir G, Anli A (1997) Pediatric uveitis in southern Turkey. Ocul Immunol Inflamm 5:197–202

    Article  CAS  PubMed  Google Scholar 

  10. Kadayifçilar S, Eldem B, Tumer B (2003) Uveitis in childhood. J Pediatr Ophthalmol Strabismus 40:335–340

    PubMed  Google Scholar 

  11. Norrsell K, Sjödell L (2008) Fuchs’ heterochromic uveitis: a longitudinal clinical study. Acta Ophthalmol 86:58–64. doi:10.1111/j.1600-0420.2007.00990.x

    Article  PubMed  Google Scholar 

  12. Kimura SJ (1978) Fuchs’ syndrome of heterochromic cyclitis in brown-eyed patients. Trans Am Ophthalmol Soc 76:76–89

    CAS  PubMed Central  PubMed  Google Scholar 

  13. Franceschetti A (1955) Heterochromic cyclitis: Fuchs’ syndrome. Am J Ophthalmol 39:50–58

    Article  CAS  PubMed  Google Scholar 

  14. Al-Mansour YS, Al-Rajhi AA, Al-Dhibi H, Abu El-Asrar AM (2010) Clinical features and prognostic factors in Fuchs’ uveitis. Int Ophthalmol 30:501–509. doi:10.1007/s10792-010-9379-7

    Article  PubMed  Google Scholar 

  15. Schwab IR (1991) The epidemiologic association of Fuchs’ heterochromic iridocyclitis and ocular toxoplasmosis. Am J Ophthalmol 111:356–362

    Article  CAS  PubMed  Google Scholar 

  16. La Hey E, Rothova A, Baarsma GS et al (1992) Fuchs’ heterochromic iridocyclitis is not associated with ocular toxoplasmosis. Arch Ophthalmol 110:806–811

    Article  PubMed  Google Scholar 

  17. Suzuki J, Goto H, Komase K et al (2010) Rubella virus as a possible etiological agent of Fuchs heterochromic iridocyclitis. Graefes Arch Clin Exp Ophthalmol 248:1487–1491. doi:10.1007/s00417-010-1434-6

    Article  PubMed  Google Scholar 

  18. de Groot-Mijnes JDF, de Visser L, Rothova A et al (2006) Rubella virus is associated with fuchs heterochromic iridocyclitis. Am J Ophthalmol 141:212–214. doi:10.1016/j.ajo.2005.07.078

    Article  PubMed  Google Scholar 

  19. Liu Y, Takusagawa HL, Chen TC, Pasquale LR (2011) Fuchs heterochromic iridocyclitis and the rubella virus. Int Ophthalmol Clin 51:1–12. doi:10.1097/IIO.0b013e31822d6923

    Article  PubMed  Google Scholar 

  20. Ruokonen PC, Metzner S, Ucer A et al (2010) Intraocular antibody synthesis against rubella virus and other microorganisms in Fuchs’ heterochromic cyclitis. Graefes Arch Clin Exp Ophthalmol 248:565–571. doi:10.1007/s00417-009-1239-7

    Article  PubMed  Google Scholar 

  21. Quentin CD, Reiber H (2004) Fuchs heterochromic cyclitis: rubella virus antibodies and genome in aqueous humor. Am J Ophthalmol 138:46–54. doi:10.1016/j.ajo.2004.02.055

    Article  PubMed  Google Scholar 

  22. Chee S-P, Jap A (2008) Presumed fuchs heterochromic iridocyclitis and Posner-Schlossman syndrome: comparison of cytomegalovirus-positive and negative eyes. Am J Ophthalmol 146:883–9.e1. doi:10.1016/j.ajo.2008.09.001

    Article  PubMed  Google Scholar 

  23. Liesegang TJ (1982) Clinical features and prognosis in Fuchs’ uveitis syndrome. Arch Ophthalmol 100:1622–1626

    Article  CAS  PubMed  Google Scholar 

  24. Jabs DA, Nussenblatt RB, Rosenbaum JT, Standardization of Uveitis Nomenclature (SUN) Working Group (2005) Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 140:509–516. doi:10.1016/j.ajo.2005.03.057

    Article  Google Scholar 

  25. Tappeiner C, Heinz C, Roesel M, Heiligenhaus A (2011) Elevated laser flare values correlate with complicated course of anterior uveitis in patients with juvenile idiopathic arthritis. Acta Ophthalmol 89:e521–e527. doi:10.1111/j.1755-3768.2011.02162.x

    Article  PubMed  Google Scholar 

  26. Heinz C, Koch JM, Zurek-Imhoff B, Heiligenhaus A (2009) Prevalence of uveitic secondary glaucoma and success of nonsurgical treatment in adults and children in a tertiary referral center. Ocul Immunol Inflamm 17:243–248. doi:10.1080/09273940902913035

    Article  PubMed  Google Scholar 

  27. Heinz C, Mingels A, Goebel C et al (2008) Chronic uveitis in children with and without juvenile idiopathic arthritis: differences in patient characteristics and clinical course. J Rheumatol 35:1403–1407

    PubMed  Google Scholar 

  28. Goble RR, Murray PI (1995) Fuchs’ heterochromic uveitis and sarcoidosis. Br J Ophthalmol 79:1021–1023

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. Smith JR, Cunningham ET (2002) Atypical presentations of ocular toxoplasmosis. Curr Opin Ophthalmol 13:387–392

    Article  PubMed  Google Scholar 

  30. Jap A, Chee S-P (2011) Viral anterior uveitis. Curr Opin Ophthalmol 22:483–488. doi:10.1097/ICU.0b013e32834be021

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest. Christoph Tappeiner is supported by an unrestricted grant from Swiss Foundation for Grants in Biology and Medicine (SFGBM)/Swiss National Science Foundation (SNSF) and Novartis Switzerland.

Author information

Authors and Affiliations

  1. Department of Ophthalmology, St. Franziskus Hospital, Hohenzollernring 74, 48145, Münster, Germany

    Christoph Tappeiner, Jens Dreesbach, Martin Roesel, Carsten Heinz & Arnd Heiligenhaus

  2. University of Essen, Essen, Germany

    Carsten Heinz & Arnd Heiligenhaus

  3. Department of Ophthalmology, Inselspital, University of Bern, Bern, Switzerland

    Christoph Tappeiner

Authors
  1. Christoph Tappeiner
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jens Dreesbach
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Martin Roesel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Carsten Heinz
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Arnd Heiligenhaus
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Arnd Heiligenhaus.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Tappeiner, C., Dreesbach, J., Roesel, M. et al. Clinical manifestation of Fuchs uveitis syndrome in childhood. Graefes Arch Clin Exp Ophthalmol 253, 1169–1174 (2015). https://doi.org/10.1007/s00417-015-2960-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-015-2960-z

Keywords

Search

Navigation