title: Successful resolution of stromal keratitis and uveitis using canakinumab in a patient with chronic infantile neurologic, cutaneous, and articular syndrome: a case study.
abstract: BACKGROUND
  Cryopyrin-associated periodic syndrome (CAPS) is a group of rare autoinflammatory diseases, and of these, chronic infantile neurologic, cutaneous, and articular/neonatal-onset multisystem inflammatory disease (CINCA/NOMID) syndrome has the most severe phenotype. Canakinumab, a monoclonal antibody that targets interleukin-1β, has been shown to be an effective treatment for resolving systemic inflammation. However, its efficacy for treating ophthalmic symptoms of this disorder remains unclear.
  
  
  RESULTS
  A 64-year-old female reported episodes of nonpruritic urticaria, fever, aseptic meningitis, and bilateral sensorineural deafness. Her son had experienced similar symptoms. She was initially referred for ophthalmologic treatment for an infectious corneal ulcer. Examination of her right eye by slit lamp biomicroscopy showed diffuse conjunctival injection, corneal infiltrates, a corneal ulcer, and hypopyon. She was therefore treated aggressively with topical and systemic antibiotics in addition to antifungal medications. However, this was ineffective. Genetic analysis detected the heterozygous germline p.Asp303Asn mutation in the NLRP3 gene in both our patient and her son. She was therefore diagnosed with CINCA/NOMID syndrome based on her clinical manifestations. All of the patient's physical and ophthalmic symptoms were resolved within a few days after the initiation of canakinumab treatment. During an 18-month follow-up period, no adverse events or severe infections were observed.
  
  
  CONCLUSIONS
  Our case report indicates that canakinumab is effective not only for the treatment of systemic inflammation but also for treating ophthalmic involvement, such as recurrent stromal keratitis and anterior uveitis.
authors.affiliation: Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. p6fb9zzp@s.okayama-u.ac.jp.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hirano
authors.affiliation: Department of Ophthalmology, Okayama Saiseikai General Hospital, Okayama, Japan. jseguchi1202@me.com.
authors.forename: Jiro
authors.initials: J
authors.lastname: Seguchi
authors.affiliation: Center for Rheumatology, Okayama Saiseikai General Hospital, Okayama, Japan. yamamura@saiseidr.jp.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamamura
authors.affiliation: Department of Ophthalmology, Okayama Saiseikai General Hospital, Okayama, Japan. akiko.n@orange.megaegg.ne.jp.
authors.forename: Akiko
authors.initials: A
authors.lastname: Narita
authors.affiliation: Department of Ophthalmology, Kurashiki Medical Center, Kurashiki, Japan. tankun@bj9.so-net.ne.jp.
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Okanobu
authors.affiliation: Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan. rnishiko@kuhp.kyoto-u.ac.jp.
authors.forename: Ryuta
authors.initials: R
authors.lastname: Nishikomori
authors.affiliation: Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan. heike@kuhp.kyoto-u.ac.jp.
authors.forename: Toshio
authors.initials: T
authors.lastname: Heike
authors.affiliation: Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. hmo529@okayama-u.ac.jp.
authors.forename: Mio
authors.initials: M
authors.lastname: Hosokawa
authors.affiliation: Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. moriza-y@okayama-u.ac.jp.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Morizane
authors.affiliation: Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. fumio327@symphony.plala.or.jp.
authors.forename: Fumio
authors.initials: F
authors.lastname: Shiraga
keywords: Canakinumab; Chronic infantile neurologic; Cryopyrin-associated periodic syndrome; Cutaneous and articular/neonatal-onset multisystem inflammatory disease syndrome; Stromal keratitis; Uveitis