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A case of periodic-fever-syndrome-like disorder with lipodystrophy, myositis, and autoimmune abnormalities

  • Case Report
  • Published:
Modern Rheumatology

Abstract

A 24-year-old Japanese woman had been suffering from a periodic fever since 10 months of age. She developed deformities in her fingers, with severe atrophy of subcutaneous adipose tissue, myositis, and frostbitten hands. She showed elevated C-reactive protein, creatine kinase, and γ-globulin. She was also positive for antinuclear, anti-DNA, anti-SS-B, and anti-U1RNP antibodies. Her myositis was similar to amyopathic dermatomyositis rather than juvenile dermatomyositis. Although consanguineous marriage of her parents and early onset of disease suggested her disease as a hereditary disorder with periodic fever, her clinical feature and laboratory tests were unlike any known periodic fever syndromes. Her disease was regarded as a unique type of periodic-fever-syndrome-like disorder with autoimmune abnormalities.

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Acknowledgments

We appreciate for the help from Dr. Shiho Kobayashi and Dr. Tsuneyo Mimori (Kyoto University Graduate School of Medicine, Kyoto, Japan) for analyzing antisynthetase antibodies.

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Authors and Affiliations

  1. Department of Clinical Pathology and Immunology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan

    Shimpei Kasagi, Seiji Kawano, Takashi Nakazawa & Shunichi Kumagai

  2. Sugino Syonika Clinic, 2-8-26 Kameyama, Asakita-Ku, Hiroshima, 731-0231, Japan

    Hirotoshi Sugino

  3. Department of Clinical Laboratory, Hyogo Medical College, 1-1 Mukogawa-cho, Nishinomiya, 663-8501, Japan

    Masahiro Koshiba

  4. First Department of Internal Medicine, Nagasaki University Hospital of Medicine and Dentistry, Graduate School of Biomedical Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan

    Kunihiro Ichinose, Hiroaki Ida & Katsumi Eguchi

Authors
  1. Shimpei Kasagi
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  2. Seiji Kawano
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  3. Takashi Nakazawa
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  4. Hirotoshi Sugino
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  5. Masahiro Koshiba
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  6. Kunihiro Ichinose
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  7. Hiroaki Ida
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  8. Katsumi Eguchi
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  9. Shunichi Kumagai
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Corresponding author

Correspondence to Shunichi Kumagai.

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Kasagi, S., Kawano, S., Nakazawa, T. et al. A case of periodic-fever-syndrome-like disorder with lipodystrophy, myositis, and autoimmune abnormalities. Mod Rheumatol 18, 203–207 (2008). https://doi.org/10.1007/s10165-008-0033-4

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  • DOI: https://doi.org/10.1007/s10165-008-0033-4

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