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Open Access Highly Accessed Research article

Roles of mast cells in the pathogenesis of inflammatory myopathy

Masaya Yokota1, Kotaro Suzuki1*, Koji Tokoyoda2, Kazuyuki Meguro1, Junichi Hosokawa1, Shigeru Tanaka1, Kei Ikeda1, Takashi Mikata3, Toshinori Nakayama24, Hitoshi Kohsaka5 and Hiroshi Nakajima1*

Author Affiliations

1 Department of Allergy and Clinical Immunology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuou-Ku, Chiba City, Chiba 260-8670, Japan

2 Department of Immunology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuou-Ku, Chiba City, Chiba 260-8670, Japan

3 Department of Neurology, National Hospital Organization Shimoshizu Hospital, 934-5, Shikato, Yotuskaido City, Chiba 284-0003, Japan

4 JST, CREST, 1-8-1 Inohana, Chuou-Ku, Chiba City, Chiba 260-8670, Japan

5 Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45. Yushima, Bunkyo-Ku, Tokyo 113-8519, Japan

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Arthritis Research & Therapy 2014, 16:R72  doi:10.1186/ar4512

Published: 17 March 2014

Abstract

Introduction

In addition to the pivotal roles of mast cells in allergic diseases, recent data suggest that mast cells play crucial roles in a variety of autoimmune responses. However, their roles in the pathogenesis of autoimmune skeletal muscle diseases have not been clarified despite their distribution in skeletal muscle. Therefore, the objective of this study is to determine the roles of mast cells in the development of autoimmune skeletal muscle diseases.

Methods

The number of mast cells in the affected muscle was examined in patients with dermatomyositis (DM) or polymyositis (PM). The susceptibility of mast cell-deficient WBB6F1-KitW/KitWv mice (W/Wv mice) to a murine model of polymyositis, C protein-induced myositis (CIM), was compared with that of wild-type (WT) mice. The effect of mast cell reconstitution with bone marrow-derived mast cells (BMMCs) on the susceptibility of W/Wv mice to CIM was also evaluated.

Results

The number of mast cells in the affected muscle increased in patients with PM as compared with patients with DM. W/Wv mice exhibited significantly reduced disease incidence and histological scores of CIM as compared with WT mice. The number of CD8+ T cells and macrophages in the skeletal muscles of CIM decreased in W/Wv mice compared with WT mice. Engraftment of BMMCs restored the incidence and histological scores of CIM in W/Wv mice. Vascular permeability in the skeletal muscle was elevated in WT mice but not in W/Wv mice upon CIM induction.

Conclusion

Mast cells are involved in the pathogenesis of inflammatory myopathy.