A subgroup of patients with necrotizing myopathy who have a novel autoantibody specificity that makes them potential candidates for immunosuppressive therapy have been identified by researchers at Johns Hopkins University School of Medicine.
The complete study is published in the September issue of
Arthritis & Rheumatism, a journal of the American College of Rheumatology.
Myopathy is a term used to describe muscle disease. The National Institute of Neurological Disorders and Stroke (NINDS) defines the inflammatory myopathies as a group of diseases that involve chronic muscle inflammation, accompanied by muscle weakness. The chronic inflammatory myopathies are idiopathic, meaning they have no known cause. They are thought to be autoimmune disorders, in which the body's white blood cells (that normally fight disease) attack blood vessels, normal muscle fibers, and connective tissue in organs, bones, and joints.
Distinguishing between immune-mediated myopathies and other necrotizing myopathies such as muscular dystrophies and self-limited toxic myopathies is crucial because only autoimmune muscle diseases respond to immunosuppressive therapy. Unfortunately, clinical evaluation and diagnostic tests sometimes fail to determine whether a necrotizing myopathy is immune mediated, resulting in undertreatment of autoimmune myopathies or inappropriate immunosuppression in patients who do not have an immune-mediated disease.
In this study, muscle biopsy specimens and serum samples were evaluated for 225 patients with myopathy who were enrolled in a longitudinal study from March 2007 through December 2008. Myopathy was defined by muscle weakness, elevated creatine kinase (CK) levels, myopathic EMG findings, muscle edema on MRI, and/or features of myopathy on muscle biopsy.