Inflammatory myopathies encompass a variety of syndromes with protean manifestations. Although the mainstay of therapy continues to include corticosteroids, there are a multitude of agents available for treating patients with myositis. These include many different immunosuppressive agents alone or in combination with each other, as well as an increasing array of novel and exciting biologic agents targeting molecules implicated in the pathogenesis of inflammatory myopathy. The systemic manifestations of myositis, particularly pulmonary involvement, are especially challenging to treat and contribute significantly to the morbidity and mortality of patients with polymyositis and dermatomyositis. Rehabilitative measures are often overlooked in the treatment of myositis, but may significantly improve the functional outcome of patients with these disorders.