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

Tadafumi Yokoyama; Masaki Shimizu; Sayaka Ishikawa; Kazuyuki Ueno; Yasuhito Hamaguchi; Kazuhiko Takehara; Akihiro Yachie

doi:10.1007/s10165-012-0627-8

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

Mod Rheumatol. 2013 Jan;23(1):167-71. doi: 10.1007/s10165-012-0627-8. Epub 2012 Mar 28.

Authors

Tadafumi Yokoyama¹, Masaki Shimizu, Sayaka Ishikawa, Kazuyuki Ueno, Yasuhito Hamaguchi, Kazuhiko Takehara, Akihiro Yachie

Affiliation

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

PMID: 22454192
DOI: 10.1007/s10165-012-0627-8

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.

Publication types

Case Reports

MeSH terms

Adolescent
Antibodies, Antinuclear / blood*
Autoantigens / immunology
B-Cell Activating Factor / blood
B-Cell Maturation Antigen / blood
B-Lymphocytes / immunology
B-Lymphocytes / pathology*
Dermatomyositis / immunology
Dermatomyositis / pathology*
Female
Humans
Immune Tolerance
Muscle, Skeletal / immunology
Muscle, Skeletal / pathology*
Nuclear Proteins / immunology*
Remission Induction
Treatment Outcome

Substances

Antibodies, Antinuclear
Autoantigens
B-Cell Activating Factor
B-Cell Maturation Antigen
Nuclear Proteins