Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr virus infection.
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 betaF1-, TCR deltaTCS1-, 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.
Uchiyama
Tomoyuki
T
Dept. of Neurology, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. uchiyama@faculty.chiba-u.jp
Arai
Kimito
K
Yamamoto-Tabata
Takako
T
Hirai
Kanji
K
Kishimoto
Kouji
K
Nakamura
Yoshiko
Y
Hattori
Takamichi
T
D000328:Adult; D000368:Aged; D002908:Chronic Disease; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis* / Q000821:virology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications / Q000175:diagnosis* / Q000821:virology*; D017285:Polymyositis / Q000150:complications / Q000175:diagnosis / Q000821:virology
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