Diagnosis and treatment of the idiopathic inflammatory myopathies

Abstract

The idiopathic inflammatory myopathies (IIMs) are rare disorders with the unifying feature of proximal muscle weakness. These diseases include polymyositis(PM), dermatomyositis (DM) and inclusion body myositis (IBM) as the most common. The diagnosis is based on the finding of weakness on exam, elevated muscles enzymes, characteristic histopathology of muscle biopsies, electromyography abnormalities and rash in DM. Myositis-specific antibodies have been helpful in defining subsets of patients with different responses to treatment and prognosis. The cornerstone of therapy is corticosteroids with the addition of other immunosuppressives in severe or refractory disease or patients with intolerable side effects. IBM is particularly difficult to treat but is more slowly progressive as compared with PM or DM. There is still a great need to find more effective and less-toxic therapies.