Ocular Involvement in Muckle-Wells Syndrome.

Abstract:

:Purpose: To present the ocular findings of the members of a family that has the diagnosis of Muckle Wells syndrome, a form of cryopyrin associated periodic syndrome (CAPS).Materials and Methods: Nine patients with MWS were included in this study. Each study participant underwent a systemic evaluation, comprehensive ophthalmic examination, and auxillary testings.Results: In this study, conjunctivitis was the most prominent ocular finding. Other relatively common ocular findings included band keratopathy, clinical signs of past uveitis, and corneal topography abnormalities. Nystagmus, corneal leukoma, and optic nerve pallor with epiretinal membrane were also detected. Rare ocular manifestations were posterior stromal corneal opacification with edema, anterior iris snychecia, and mild cataract.Conclusion: MWS is a rare systemic autoinflammatory disorder that presents with a variety of ocular findings. Exacerbation of systemic and ocular findings with cold is a hallmark of the disease.

journal_name

Ocul Immunol Inflamm

authors

Cekic S,Yalcinbayir O,Kilic SS

doi

10.1080/09273948.2018.1552305

subject

Has Abstract

pub_date

2020-01-01 00:00:00

pages

70-78

issue

1

eissn

0927-3948

issn

1744-5078

journal_volume

28

pub_type

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