Disease models of myositis: overview of cell culture and rodent systems.

Abstract

Inflammatory myopathies (in short: myositis) display a heterogenic group of rare inflammatory diseases of the skeletal muscle and other organs such as lung, heart and skin. Patients typically display muscular weakness, wasting and a variable response to treatment. The pathogenesis involves invasion of muscle fibres by mononuclear cells and deposition of autoantibodies. In vitro and in vivo models are crucial to understand the so far unresolved complex network of pathomechanisms and how to design future treatment strategies. So far, no model can represent all features of the human disease, but each facilitates analysis of distinct mechanisms of the disease. A range of different in vitro and in vivo models have been developed in recent years to functionally study myositis pathology. This review provides an overview of muscle cell culture systems and transgenic as well as inducible animal models that each represent distinct features of myositis.