Inflammatory myopathies: Evaluation and management

被引:38
作者
Greenberg, Steven A. [1 ,2 ,3 ,4 ]
机构
[1] Brigham & Womens Hosp, Dept Neurol, Div Neuromusc Dis, Boston, MA 02115 USA
[2] Harvard Univ, Sch Med, Cambridge, MA 02138 USA
[3] Childrens Hosp, Informat Program, Boston, MA 02115 USA
[4] Harvard Univ, MIT, Div Hlth Sci & Technol, Boston, MA 02115 USA
关键词
dermatomyositis; inclusion body myositis; inflammatory myopathy; necrotizing myopathy; polymyositis;
D O I
10.1055/s-2008-1062267
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The inflammatory myopathies, including dermatomyositis, inclusion body myositis, and polymyositis, are poorly understood autoimmune diseases affecting skeletal muscle. Dermatomyositis is a disease mainly of skin and muscle, but may affect lung and other tissues. Proximal or generalized weakness or skin rash are the typical presenting features. Inclusion body myositis has a specific clinical pattern of weakness that generally distinguishes it from other inflammatory myopathies, with prominent involvement of wrist and finger flexors, and quadriceps. Polymyositis generally presents with proximal or generalized weakness. Typical dermatomyositis muscle pathology is quite distinct, with perivascular inflammatory cells that include plasmacytoid dendritic cells, and abnormal capillaries and perimysial perifascicular myofibers. Both inclusion body myositis and polymyositis usually have infiltration into muscle of large numbers of inflammatory cells, typically surrounding and displacing, and sometimes invading, myofibers. Inclusion body myositis is refractory to corticosteroids and to several immunomodulating therapies that have been used. Dermatomyositis and polymyositis are treated with corticosteroids and a variety of agents. Osteoporosis and opportunistic infections pose a significant risk during treatment of patients. This review discusses the clinical manifestations, pathology, and treatment approaches for the inflammatory myopathies.
引用
收藏
页码:241 / 249
页数:9
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