Salvarani Carlo, Cantini Fabrizio, Hunder Gene G
Unit of Rheumatology, Arcispedale S Maria Nuova, Reggio Emilia, Italy.
Lancet. 2008 Jul 19;372(9634):234-45. doi: 10.1016/S0140-6736(08)61077-6.
Polymyalgia rheumatica and giant-cell arteritis are closely related disorders that affect people of middle age and older. They frequently occur together. Both are syndromes of unknown cause, but genetic and environmental factors might have a role in their pathogenesis. The symptoms of polymyalgia rheumatica seem to be related to synovitis of proximal joints and extra-articular synovial structures. Giant-cell arteritis primarily affects the aorta and its extracranial branches. The clinical findings in giant-cell arteritis are broad, but commonly include visual loss, headache, scalp tenderness, jaw claudication, cerebrovascular accidents, aortic arch syndrome, thoracic aorta aneurysm, and dissection. Glucocorticosteroids are the cornerstone of treatment of both polymyalgia rheumatica and giant-cell arteritis. Some patients have a chronic course and might need glucocorticosteroids for several years. Adverse events of glucocorticosteroids affect more than 50% of patients. Trials of steroid-sparing drugs have yielded conflicting results. A greater understanding of the molecular mechanisms involved in the pathogenesis should provide new targets for therapy.
风湿性多肌痛和巨细胞动脉炎是密切相关的疾病，影响中年及老年人群。它们常同时发生。两者均为病因不明的综合征，但遗传和环境因素可能在其发病机制中起作用。风湿性多肌痛的症状似乎与近端关节滑膜炎及关节外滑膜结构有关。巨细胞动脉炎主要累及主动脉及其颅外分支。巨细胞动脉炎的临床表现广泛，但通常包括视力丧失、头痛、头皮压痛、颌部间歇性运动障碍、脑血管意外、主动脉弓综合征、胸主动脉瘤和夹层。糖皮质激素是治疗风湿性多肌痛和巨细胞动脉炎的基石。一些患者病程呈慢性，可能需要使用糖皮质激素数年。超过50%的患者会出现糖皮质激素的不良事件。类固醇节省药物的试验结果相互矛盾。对发病机制中涉及的分子机制有更深入的了解应能为治疗提供新的靶点。
