Successful resolution of stromal keratitis and uveitis using canakinumab in a patient with chronic infantile neurologic, cutaneous, and articular syndrome: a case study

Masayuki Hirano, Jiro Seguchi, Masahiro Yamamura, Akiko Narita, Hirotaka Okanobu, Ryuta Nishikomori, Toshio Heike, Mio Hosokawa, Yuki Morizane, Fumio Shiraga

Research output: Contribution to journalArticle

7 Citations (Scopus)

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. Findings: 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.

Original languageEnglish
Article number34
Pages (from-to)1-5
Number of pages5
JournalJournal of Ophthalmic Inflammation and Infection
Volume5
Issue number1
DOIs
Publication statusPublished - Dec 1 2015

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Cryopyrin-Associated Periodic Syndromes
Keratitis
Uveitis
Corneal Ulcer
Nuclear Family
Nervous System
Joints
Inflammation
Aseptic Meningitis
Anterior Uveitis
Skin
Urticaria
Deafness
Therapeutics
Rare Diseases
Interleukin-1
Fever
Monoclonal Antibodies
Anti-Bacterial Agents
Phenotype

Keywords

  • Canakinumab
  • Chronic infantile neurologic
  • Cryopyrin-associated periodic syndrome
  • Cutaneous and articular/neonatal-onset multisystem inflammatory disease syndrome
  • Stromal keratitis
  • Uveitis

ASJC Scopus subject areas

  • Ophthalmology
  • Infectious Diseases

Cite this

Successful resolution of stromal keratitis and uveitis using canakinumab in a patient with chronic infantile neurologic, cutaneous, and articular syndrome : a case study. / Hirano, Masayuki; Seguchi, Jiro; Yamamura, Masahiro; Narita, Akiko; Okanobu, Hirotaka; Nishikomori, Ryuta; Heike, Toshio; Hosokawa, Mio; Morizane, Yuki; Shiraga, Fumio.

In: Journal of Ophthalmic Inflammation and Infection, Vol. 5, No. 1, 34, 01.12.2015, p. 1-5.

Research output: Contribution to journalArticle

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AU - Hirano, Masayuki

AU - Seguchi, Jiro

AU - Yamamura, Masahiro

AU - Narita, Akiko

AU - Okanobu, Hirotaka

AU - Nishikomori, Ryuta

AU - Heike, Toshio

AU - Hosokawa, Mio

AU - Morizane, Yuki

AU - Shiraga, Fumio

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