Vol.23 No.3

Review Article

Guidance on the use of canakinumab in patients with cryopyrin-associated periodic syndrome in Japan

Authors

Shumpei Yokota1 , Ryuta Nishikomori2 , Hidetoshi Takada3 , Masako Kikuchi1 , Tomo Nozawa1 , Taichi Kanetaka1 , Toshitaka Kizawa1 , Takako Miyamae 1 , Masaaki Mori 1 , Toshio Heike2 , Toshiro Hara3 , Tomoyuki Imagawa1

  • Department of Pediatrics, Yokohama City University School of Medicine, 3-9, Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan
  • Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan
  • Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Received:

18 June 2012

Accepted:

5 September 2012

Published online:

20 October 2012

Full Text

PDF　(member's only)

Abstract

Cryopyrin-associated periodic syndrome (CAPS) is an orphan disease with incidence of about one in 1,000,000 persons. This autoinflammatory disease develops in the neonatal period or early childhood, with various inflammatory symptoms occurring repeatedly throughout the patient’s lifetime. It is caused by abnormality of the NLRP3 protein which mediates the intracellular signal transduction mechanism of inflammatory processes, resulting in continuous overproduction of interleukin (IL)-1β, which induces chronic inflammation and progressive tissue damage. Definitive diagnosis of CAPS is difficult, and treatment has also been difficult because of a lack of effective medications in Japan. Clinical studies of human anti-human IL-1β monoclonal antibody (canakinumab) treatment were conducted in Japan, and approval was granted for therapeutic use of canakinumab for CAPS in September 2011. Similar to other biological drugs, canakinumab is clinically highly effective. However, sufficient attention to the method of use and adverse drug reactions is necessary. This guidance describes the use of canakinumab in Japan for CAPS in relation to exclusion criteria, method of use, evaluation criteria, and adverse drug reactions.

Key words

Canakinumab, Cryopyrin-associated periodic syndrome, Human anti-human IL-1β monoclonal antibody, Interleukin-1β