Inclusion body myositis in Connecticut - Observations in 35 patients during an 8-year period
Inclusion body myositis in Connecticut - Observations in 35 patients during an 8-year period
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DOI：
10.1097/00005792-200109000-00006
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发表时间：
2001-09-01
期刊：
影响因子：
1.6
通讯作者：
North, WA
中科院分区：
文献类型：
--
作者：
Felice, KJ;North, WA
散发性包涵体肌炎（IBM）是50岁以上患者中最常见的获得性肌病（24，30）。自最早的病理描述以来，人们对这种疾病的临床和病因学特征有了很多了解（2，9）。临床上，大多数患者在一段时间后出现缓慢进行性肢体无力。发病时虚弱和萎缩的主要部位包括股四头肌、长指屈肌和踝伸肌（23，24）。IBM的诊断在组织学上通过与镶边空泡和管状丝状包涵体相关的炎性肌病的发现来确定（15）。其他病理特征包括β-淀粉样蛋白及其前体蛋白、过度磷酸化tau、α1-抗胰凝乳蛋白酶、载脂蛋白E、泛素和朊病毒蛋白的异常积聚（2，29）。最初，IBM的特点和治疗作为一个原发性炎症性肌病;然而，异常细胞蛋白质的积累和疾病症状对各种免疫疗法的难治性已经提高了IBM是肌肉变性疾病的可能性（29）。自从1967年IBM的第一个临床病理学描述（9）以来，已经报道了描述IBM的临床和病理特征的几个系列（1，5，7，8，11，12，14，18，20，21，26）。然而，报告患病率，转诊模式，肌肉无力的具体分布在介绍，和疾病进展率的大型区域性研究普遍缺乏。在本研究中，我们回顾性地研究了这些功能，在一系列的IBM患者在康涅狄格州的区域转诊中心超过8年。
Sporadic inclusion body myositis (IBM) is the most common acquired myopathy in patients older than 50 years (24, 30). Since the earliest pathologic descriptions, much has been learned about the clinical and etiopathologic features of this disorder (2, 9). Clinically, most patients present to their physicians following a period of slowly progressive limb weakness. The predominant sites of weakness and atrophy at onset include the quadriceps, long finger flexors, and ankle extensors (23, 24). The diagnosis of IBM is established histologically by the findings of an inflammatory myopathy associated with rimmed vacuoles and tubulofilamentous inclusions (15). Other pathologic features include abnormal accumulations of β-amyloid and its precursor proteins, hyperphosphorylated tau, α1-antichymotrypsin, apolipoprotein E, ubiquitin, and prion protein (2, 29). Initially, IBM was characterized and treated as a primary inflammatory myopathy; however, the accumulation of abnormal cellular proteins and the refractoriness of the disease symptoms to the various immunotherapies have raised the possibility that IBM is a degenerative disease of muscle (29).Since the first clinicopathologic description of IBM in 1967 (9), several series describing the clinical and pathologic features of IBM have been reported (1, 5, 7, 8, 11, 12, 14, 18, 20, 21, 26). However, large regional studies reporting the prevalence rate, referral patterns, specific distribution of muscle weakness at presentation, and rate of disease progression have generally been lacking. In the present study we examine these features retrospectively in a series of patients with IBM seen in a regional referral center in Connecticut over 8 years.