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Autoimmune myositis (AIM) is a group of uncommon diseases that primarily involves the skeletal muscles. These diseases include polymyositis, anti-synthetase syndrome, dermatomyositis, juvenile dermatomyositis, inclusion body myositis, necrotizing myopathy and overlap syndromes such as mixed connective tissue disease and scleromyositis. The main symptom is progressive muscle weakness, especially of the muscles closest to the torso. Each AIM subset is associated with a unique autoantibody biomarker that can help the physician make an accurate diagnosis and initiate appropriate treatment. A small proportion of AIM patients have or eventually develop cancer and several autoantibodies are associated with a higher risk of cancer.