Animal models of myositis

Abstract

Current animal models of human myositis include spontaneous, induced, and transgenic models. Although it is clear that none of these models possesses all the features of the human diseases, they may provide insight into the pathophysiologic mechanisms, and possibly the therapy, of inflammatory muscle disease. Because the human IIMs are phenotypically heterogeneous, but may be divided into more homogeneous subgroups based upon clinical or serologic features, it is possible that different pathogeneses are involved in different subgroups. It is unlikely that any single model would reproduce all features of the human disease. It may be possible, however, to gain insight into some subgroups of the human disease if certain animal models faithfully reproduce one or more subtypes or aspects of the IIMs. Because immunogenetic risk factors, and exposure to certain environmental agents important in triggering myositis in genetically susceptible persons, may be necessary components for human disease induction, transgenic approaches to humanizing murine immune systems and a better understanding of environmental risk factors will be productive avenues for future research. Additional investigations into the molecular basis of the human myositis syndromes and the pathogenesis of the spontaneous, induced, and transgenic animal models should ultimately allow for better understanding and therapy of these diseases.