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An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection
09:43

An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection

Published on: March 30, 2018

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

Tomoyuki Uchiyama1, Kimito Arai, Takako Yamamoto-Tabata

  • 1Dept. of Neurology, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. uchiyama@faculty.chiba-u.jp

Journal of Neurology
|March 3, 2005
PubMed
Abstract

Insights

Chronic active Epstein-Barr virus infection (CAEBV) can cause a new form of chronic generalized myositis. This condition mimics polymyositis and requires specific diagnostic investigation, especially with lingual or orbital involvement.

Area of Science:

  • Immunology
  • Neurology
  • Virology

Background:

  • Chronic generalized myositis is a newly identified complication of chronic active Epstein-Barr virus infection (CAEBV).
  • This condition presents clinically similar to polymyositis but has distinct pathological features.

Observation:

  • Three patients with CAEBV presented with chronic generalized myositis, two with initial proximal weakness and muscle atrophy, and one with late-stage generalized myositis.
  • Lingual and/or orbital myositis were observed in some patients, differentiating it from typical polymyositis.
  • Immunotherapy proved ineffective, and the prognosis was poor across all cases.

Findings:

  • Muscle biopsies revealed infiltrating lymphocytes characterized as CD45RO+, CD3+, CD4-, CD8-, TCR betaF1-, TCR deltaTCS1-, CD56-, CD79a-, CD21-, HLA-DR+, ZEBRA-, LMP1-, and EBER+ T cells.
  • Evidence of oligoclonal expansion of Epstein-Barr virus-infected T cells was found within the muscle tissue.
  • No malignant lymphocytes were detected, ruling out a neoplastic process.

Implications:

  • This distinct myositis entity necessitates differentiation from polymyositis and other conventional myopathies.
  • Clinicians should consider investigating for occult CAEBV in patients with steroid-nonresponsive chronic progressive generalized myositis, particularly those with lingual or orbital involvement.

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