Vol.23 No.1

Case Report

Accumulation of mature B cells in the inflamed muscle tissue of a patient with anti-155/140 antibody-positive juvenile dermatomyositis

Authors

Tadafumi Yokoyama1 , Masaki Shimizu1 , Sayaka Ishikawa1 , Kazuyuki Ueno1 , Yasuhito Hamaguchi2 , Kazuhiko Takehara2 , Akihiro Yachie1

  • Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, 920-8641, Japan
  • Department of Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan
Received:

13 December 2011

Accepted:

28 February 2012

Published online:

28 March 2012

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Abstract

We report the first case of a Japanese patient with anti-155/140 antibody-positive juvenile dermatomyositis (JDM). Her clinical features included severe cutaneous involvement. Serum B cell-activating factor levels were significantly increased. Mature class-switched memory B cells accumulated in inflamed muscle tissue but decreased in peripheral blood. These findings indicate that loss of B cell tolerance and accumulation of mature B cells in inflamed muscle tissue play an important role in the pathogenesis of JDM.

Key words

Anti-155/140 antibody - B cells - Juvenile dermatomyositis