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Induction of Ocular Surface Inflammation and Collection of Involved Tissues
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Ocular Involvement in Muckle-Wells Syndrome.
Sukru Cekic1, Ozgur Yalcinbayir2, Sara Sebnem Kilic1
1Pediatric Allergy and Clinical Immunology, Uludag University School of Medicine, Bursa, Turkey.
Ocular Immunology and Inflammation
|December 18, 2018
Summary
Muckle Wells syndrome (MWS), a cryopyrin-associated periodic syndrome, commonly causes ocular issues like conjunctivitis and band keratopathy. Cold exposure can worsen these systemic and eye symptoms in MWS patients.
Area of Science:
- Ophthalmology
- Rheumatology
- Genetics
Background:
- Muckle Wells syndrome (MWS) is a rare autoinflammatory disorder.
- It is a subtype of cryopyrin-associated periodic syndromes (CAPS).
- Ocular manifestations in MWS are not well-documented.
Purpose of the Study:
- To document the ocular findings in patients diagnosed with Muckle Wells syndrome.
- To characterize the spectrum of eye abnormalities associated with MWS.
Main Methods:
- Systemic evaluation of nine MWS patients.
- Comprehensive ophthalmic examinations.
- Auxiliary testing for ocular assessment.
Main Results:
- Conjunctivitis was the most frequent ocular finding.
- Common findings included band keratopathy, signs of past uveitis, and corneal topography abnormalities.
- Less common findings included nystagmus, corneal leukoma, optic nerve pallor, and epiretinal membrane.
Conclusions:
- Muckle Wells syndrome presents with diverse ocular manifestations.
- Cold exposure is a key trigger for both systemic and ocular symptoms in MWS.
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