title: First report of keratitis in familial cold autoinflammatory syndrome.
abstract: OBJECTIVE
  To report corneal manifestations of familial cold autoinflammatory syndrome (FCAS) for the first time.
  
  
  METHODS
  small case series
  
  
  METHODS
  Medical records of three members of a single family were reviewed after obtaining institutional review board (IRB) approval and informed consent.
  
  
  METHODS
  All three members presented with a long history of maculopapular rash after cold exposure starting in childhood associated with nausea, low-grade fever, fatigue and arthralgia that lasted less than 24 hours. Their ocular manifestations consisted of ocular pain, photophobia and keratitis with subsequent stromal haziness.
  
  
  RESULTS
  Patients underwent systemic therapy with canalinumab (Ilaris). They responded very well to repeated injections of Ilaris without side effects.
  
  
  CONCLUSIONS
  FCAS causes lifelong debilitating effects that restrict patients' daily activities. Ilaris is an FDA-approved treatment for this condition and that typically results in dramatic improvement in clinical and laboratory measures of inflammation, and is well tolerated. Our report is the first small case series of FCAS with keratitis that responded to Ilaris beautifully.
authors.affiliation: Duke University Eye Center, Durham, N.C.. Electronic address: ladan.espandar@duke.edu.
authors.forename: Ladan
authors.initials: L
authors.lastname: Espandar
authors.affiliation: Duke University Eye Center, Durham, N.C.
authors.forename: Christopher S
authors.initials: CS
authors.lastname: Boehlke
authors.affiliation: Duke University Eye Center, Durham, N.C.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Kelly
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D003080:Cold Temperature / Q000009:adverse effects; D056587:Cryopyrin-Associated Periodic Syndromes / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D058447:Eye Pain / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006801:Humans; D053583:Interleukin-1beta / Q000037:antagonists & inhibitors; D007634:Keratitis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D020795:Photophobia / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D053583:Interleukin-1beta; C541220:canakinumab