Journal
CORNEA
Volume 35, Issue 12, Pages 1656-1657Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/ICO.0000000000000951
Keywords
PLAID; IL-1 beta; cornea; inflammation
Categories
Funding
- NEI NIH HHS [K12 EY016335] Funding Source: Medline
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Purpose: To report the ocular manifestations of phospholipase-C gamma 2-associated antibody deficiency and immune dysregulation (PLAID). Methods: Case report and literature review. Results: A 21-year-old woman diagnosed with PLAID was referred for evaluation of repeated episodes of ocular inflammation resulting in bilateral peripheral corneal pannus with episcleritis and corneal scarring accompanied by systemic manifestations including epidermolysis bullosa and interstitial lung disease. Systemic immunosuppression with corticosteroids and interleukin-1 (IL-1) receptor antagonist (anakinra) was supplemented with topical anakinra to avoid systemic side effects, which resulted in partial improvement of the ocular symptoms. Oral prednisone was restarted to treat active lesions during bouts of inflammation. Conclusions: Ocular PLAID is a bilateral chronic or recurrent inflammatory disease of the ocular surface leading to severe and early cicatricial ocular surface and corneal involvement because of high IL-1 production. Management of PLAID may require both topical and systemic immunomodulatory treatments, potentially including targeted local anti-IL-1 therapy.
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