The Inflammatory Myopathies

The inflammatory myopathies are a group of autoimmune diseases characterized by progressive skeletal muscle weakness associated with inflammatory cell infiltration within the muscle. The muscle tissue of patients with inclusion body myositis (IBM) contains an immune infiltrate that had previously been uncharacterized. To clarify the contributions of autoimmunity to IBM, my group and I reported for the first time that plasma cells populate the muscle tissue of patients with myositis. This led to the successful identification of several novel antigen targets of these cells by our group and others, suggesting that autoimmunity plays a role in IBM. We are currently pursuing this work in a clinicopathological study using clinical data and our assay results to better understand the associations between the clinical presentations of IBM and the autoimmune process.

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Ray, A., Amato, A.A., Bradshaw, E.M., Felice, K.J., DiCapua, D.B., Goldstein, J.M., Lundberg, I.E., Nowak, R.J., Ploegh, H.L., Spooner, E., Wu, Q., Willis, S.N., and O'Connor, K.C. (2012). Autoantibodies produced at the site of tissue damage provide evidence of humoral autoimmunity in inclusion body myositis. PLoS One, 7(10), e46709. PMCID: PMC3465259.

Paltiel, A.D., Ingvarsson, E., Lee, D.K., Leff, R.L., Nowak, R.J., Petschke, K.D., Richards-Shubik, S., Zhou, A., Shubik, M., and O'Connor, K.C. (2015). Demographic and clinical features of inclusion body myositis in North America. Muscle Nerve, 52(4):527-533. PMCID: PMC4869122.