Article in Japanese

Clinical and immunological aspects of 22 cases of PFAPA syndrome

Risa TANAKA, Nazuna HONMA, Tadamasa TAKANO, Tsutomu OH-ISHI, Kosuke JOH

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) is a disorder of childhood characterized by periodic occurrences of fever, aphthous stomatitis, pharyngitis, and cervical adenitis, but the causes of clinical signs such as fever and the gene responsible for the disease have not yet been explained. In this study, a comparison was made of the serum proinflammatory cytokine-concentrations between PFAPA (22 cases), Kawasaki disease (KD), which is an acute febrile disease of childhood with a distinctive vasculitis and the self-limited nature of the illness, and the reference value from healthy controls. During PFAPA, only interleukin-6 (IL-6) rose significantly accompanying the subtle increment of TNF-α, compared with the defervescence of PFAPA or healthy control, while IL-6, TNF-α, and IL-10 increased in the febrile period of KD. The serum levels of IL-6, TNF-α, and IL-10 were significantly higher in the febrile period of KD than with PFAPA. The HLA-DR expression on T-cells, which is thought of as T-cell activation, did not increase in an episode compared with the afebrile period of PFAPA, and a left shift of neutrophils was uncommon both in PFAPA and in KD. It was considered that fever and fatigue as the major manifestation of PFAPA was correlated to a rise of the serum IL-6 concentration. The mechanism of increased IL-6 production will have to be elucidated. Furthermore, administration of the histamine H₂-receptor antagonist (H₂-blocker), cimetidine, was effective for the prevention and treatment in 70% of patients with PFAPA, and so the affirmative use of H₂-blocker is indicated. It should be essential to reveal the mode of action of the H₂-blocker on the relief of fever due to PFAPA.

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Division of Infectious Disease, Immunology, and Allergy, Saitama Children's Medical Center

Key words
Received	September 7, 2011
Accepted	December 20, 2011

24 (2)：155─161,2012