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Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr virus infection

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Abstract

Background

Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr virus infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV.

Methods

To clarify the pathological character of this myositis, we investigated the distribution, clonality, and the immunophenotype of EBV–infected cells and lymphocytes infiltrating in muscles.

Results

Clinically, two patients showed symmetrical proximal weakness and muscle atrophy as the initial and main symptom. Although the condition resembled polymyositis, they had also lingual and/or orbital myositis. The other patient showed generalized myositis at the late phase of CAEBV. In all of them, immunotherapy was ineffective and prognosis was poor. Intramuscular infiltrating lymphocytes in our patients were mainly CD45RO+, CD3+, CD4–, CD8–, TCR βF1–, TCR δTCS1–, CD56–, CD79a–, CD21–, HLA-DR+, ZEBRA –, LMP1–, and EBER+ T cells. Oligoclonal expansion of EBV–infected T cells was shown in the muscles. However, there were no malignant lymphocytes.

Conclusions

This new form of myositis must be distinguished from polymyositis and the other conventional forms of myositis. Careful investigation of hidden CAEBV is recommended when patients present with steroid non–responsive chronic progressive generalized myositis, in particular, with lingual or orbital involvement.

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Authors and Affiliations

  1. Dept. of Neurology, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan

    Tomoyuki Uchiyama MD, Kimito Arai MD & Takamichi Hattori MD

  2. Dept. of Stomatology, University of California, San Francisco, USA

    Takako Yamamoto-Tabata PhD

  3. Dept. of Tumor Virology, Division of Virology and Immunology, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan

    Kanji Hirai† PhD

  4. Dept. of Pathology, Showa University Fujigaoka Hospital, Fujigaoka, Japan

    Kouji Kishimoto

  5. Dept. of Clinical Pathology, Showa University Fujigaoka Hospital, Fujigaoka, Japan

    Yoshiko Nakamura MD

Authors
  1. Tomoyuki Uchiyama MD
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  2. Kimito Arai MD
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  3. Takako Yamamoto-Tabata PhD
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  4. Kanji Hirai† PhD
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  5. Kouji Kishimoto
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  6. Yoshiko Nakamura MD
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  7. Takamichi Hattori MD
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Correspondence to Tomoyuki Uchiyama MD.

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Uchiyama, T., Arai, K., Yamamoto-Tabata, T. et al. Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr virus infection. J Neurol 252, 519–525 (2005). https://doi.org/10.1007/s00415-005-0679-1

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  • DOI: https://doi.org/10.1007/s00415-005-0679-1

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