title: Cryopyrin-associated periodic syndromes and the eye.
abstract: OBJECTIVE
  To describe the ophthalmologic findings in two patients with Muckle-Wells Syndrome, a phenotype of the Cryopyrin Associated Periodic Syndromes (CAPS) spectrum. There is currently sparse ophthalmic literature regarding the ocular manifestations of CAPS. We hope to increase awareness of this spectrum of diseases and the importance of proper treatment amongst eye care professionals.
  
  
  METHODS
  Interventional Case Series.
  
  
  RESULTS
  Patient 1 experienced resolution of aseptic meningitis, papilledema, and anterior uveitis following treatment with anikinra. Patient 2 experienced resolution of panuveitis following treatment with anikinra.
  
  
  CONCLUSIONS
  The severe ocular manifestations of the most severe CAPS phenotype, Chronic Infantile Neurological Cutaneous and Articular Syndrome/Neonatal Onset Multisystem Inflammatory Disease Syndrome (CINCA/NOMID) have been previously described. There is increasing evidence that patients may experience similar ocular disease with the milder phenotype of Muckle-Wells Sydnrome. There is also increasing evidence that appropriate therapy can have a profound effect on patient prognosis.
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Oberg
authors.forename: Albert T
authors.initials: AT
authors.lastname: Vitale
authors.forename: Robert O
authors.initials: RO
authors.lastname: Hoffman
authors.forename: John F
authors.initials: JF
authors.lastname: Bohnsack
authors.forename: Judith E
authors.initials: JE
authors.lastname: Warner
mesh_terms: D000328:Adult; D002675:Child, Preschool; D056587:Cryopyrin-Associated Periodic Syndromes / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D010211:Papilledema / Q000175:diagnosis / Q000209:etiology*; D014606:Uveitis, Anterior / Q000175:diagnosis / Q000209:etiology*