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Markedly elevated CD64 expressions on neutrophils and monocytes are useful for diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome during flares

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Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most commonly encountered autoinflammatory disease in children, but its pathogenesis and diagnostic biomarkers are unknown. In this study, we examined the utility of CD64, a member of the Fcγ receptors, expressions on neutrophils and monocytes in diagnosing patients with PFAPA, along with other autoinflammatory diseases exhibiting periodic fever, and bacterial infections. Although CD64 was expressed at a similar level in the attack-free period of PFAPA and in controls, CD64 expressions on both neutrophils and monocytes were dramatically increased during attacks. Serum IFN-γ also increased in some PFAPA patients during flares, suggesting the involvement of T cell activation. Our findings demonstrate that remarkable CD64 expression during PFAPA flares serves as a potential biomarker for the diagnosis. We also suspect that IFN-γ, possibly from retention of activated T cells in peripheral tissues, increases CD64 synthesis in such cases.

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Abbreviations

PFAPA:

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

IL:

Interleukin

IFN-γ:

Interferon γ

TNF-α:

Tumor necrosis factor alpha

IP-10:

Interferon gamma-induced protein 10

CXCL10/9:

C-X-C motif ligand 10/9

MIG:

Monokine induced by gamma interferon

G-CSF:

Granulocyte colony-stimulating factor

Fcγ-Rs:

Fcγ receptors

NK:

Natural killer

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Conflict of interest

All the authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Infection and Host Defense, Shinshu University Graduate School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Takashi Yamazaki, Sho Hokibara & Kazunaga Agematsu

  2. Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

    Takashi Yamazaki

  3. Department of Pediatrics, Suwa Red Cross Hospital, Suwa, Japan

    Sho Hokibara

  4. Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan

    Tomonari Shigemura & Norimoto Kobayashi

  5. Children’s Medical Center, Northern Yokohama Hospital, Showa University, Yokohama, Japan

    Kimiko Honda, Yoh Umeda & Kazunaga Agematsu

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  1. Takashi Yamazaki
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  2. Sho Hokibara
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  4. Norimoto Kobayashi
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  7. Kazunaga Agematsu
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Correspondence to Kazunaga Agematsu.

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Yamazaki, T., Hokibara, S., Shigemura, T. et al. Markedly elevated CD64 expressions on neutrophils and monocytes are useful for diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome during flares. Clin Rheumatol 33, 677–683 (2014). https://doi.org/10.1007/s10067-014-2542-1

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  • DOI: https://doi.org/10.1007/s10067-014-2542-1

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