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包涵体肌炎的谱系

Spectrum of inclusion body myositis.

作者信息

Ringel S P, Kenny C E, Neville H E, Giorno R, Carry M R

机构信息

Department of Neurology, University of Colorado Health Sciences Center, Denver 80262.

出版信息

Arch Neurol. 1987 Nov;44(11):1154-7. doi: 10.1001/archneur.1987.00520230042011.

DOI:10.1001/archneur.1987.00520230042011
PMID:2823752
Abstract

The clinical, laboratory, and biopsy features are described for a large group of patients with inclusion body myositis (IBM) (15 men and four women; mean age, 63 years). A quantitative histopathologic analysis of muscle biopsy specimens revealed less fiber necrosis and endomysial and perivascular inflammation in IBM than in polymyositis (PM) and dermatomyositis, but a more frequent occurrence of dark-angular and hypertrophied fibers. Rimmed vacuoles were present in 3.4% of all fibers and 15- to 18-nm filaments were identified in the biopsy specimens of nine of 11 patients. A panel of monoclonal antibodies immunoreactive with lymphocytes and cells of monocyte/macrophage lineage suggested that the inflammatory reaction in IBM was similar to that in PM (but not dermatomyositis) and mediated by cellular immune responses. These studies confirm the clinical and histopathologic distinctions between IBM and chronic PM, and that differentiation between these disorders is often difficult.

摘要

描述了一大组包涵体肌炎(IBM)患者(15名男性和4名女性;平均年龄63岁)的临床、实验室及活检特征。对肌肉活检标本进行的定量组织病理学分析显示,与多发性肌炎(PM)和皮肌炎相比,IBM的纤维坏死、肌内膜及血管周围炎症较少,但暗角形和肥大纤维的出现更为频繁。边缘空泡存在于所有纤维的3.4%中,在11例患者中的9例活检标本中鉴定出15至18纳米的细丝。一组与淋巴细胞及单核细胞/巨噬细胞系细胞发生免疫反应的单克隆抗体表明,IBM中的炎症反应与PM(而非皮肌炎)中的相似,且由细胞免疫反应介导。这些研究证实了IBM与慢性PM在临床和组织病理学上的区别,且这些疾病之间的鉴别往往困难。

相似文献

1
Spectrum of inclusion body myositis.包涵体肌炎的谱系
Arch Neurol. 1987 Nov;44(11):1154-7. doi: 10.1001/archneur.1987.00520230042011.
2
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