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Retinal vasculitis associated with CREST syndrome.

Sungjae Yang1,2, Laura J Kopplin3, James T Rosenbaum2,4,5

  • 1Department of Ophthalmology, Gangneung Asan Hospital, Ulsan University, Gangneung, South Korea.

American Journal of Ophthalmology Case Reports
|May 22, 2018
PubMed
Summary

This study highlights retinal vasculitis as a new ocular complication of CREST syndrome. Early immunomodulatory treatment may help prevent vision loss in affected patients.

Keywords:
CREST syndromeRetinal vasculitis

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Area of Science:

  • Ophthalmology
  • Rheumatology
  • Immunology

Background:

  • CREST syndrome is a rare autoimmune disorder affecting connective tissues.
  • Ocular manifestations of CREST syndrome are not well-documented.
  • Retinal vasculitis is an inflammation of retinal blood vessels.

Observation:

  • Two patients with CREST syndrome presented with distinct ocular inflammatory conditions.
  • Patient 1 exhibited unilateral panuveitis with significant retinal vasculitis and prior uveitis.
  • Patient 2 experienced a branch retinal artery occlusion and bilateral retinal vasculitis.

Findings:

  • Retinal vasculitis is a previously unreported ocular finding in patients with CREST syndrome.
  • Both patients demonstrated inflammatory ocular disease requiring systemic treatment.

Implications:

  • Retinal vasculitis should be considered in the differential diagnosis of ocular inflammation in patients with CREST syndrome.
  • Prompt initiation of immunomodulatory therapy, such as prednisone and mycophenolate mofetil, may be crucial in mitigating ocular morbidity.
  • This finding expands the spectrum of known ocular complications associated with CREST syndrome.