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主要组织相容性复合体I类表达可作为一种诊断工具,用于区分特发性炎性肌病和肌营养不良症。

Major histocompatibility complex class I expression can be used as a diagnostic tool to differentiate idiopathic inflammatory myopathies from dystrophies.

作者信息

Sundaram C, Uppin Megha S, Meena A K

机构信息

Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India.

出版信息

Neurol India. 2008 Jul-Sep;56(3):363-7. doi: 10.4103/0028-3886.43457.

DOI:10.4103/0028-3886.43457
PMID:18974565
Abstract

AIM

Utility of major histocompatibility complex (MHC) Class I antigen immunostaining was studied to differentiate idiopathic inflammatory myopathies from dystrophies.

MATERIALS AND METHODS

Forty muscle biopsies including seven dermatomyositis (DM), six polymyositis (PM), two sporadic inclusion body myositis (sIBM), 20 dystrophies (one Duchenne, three Becker's, four alpha, one gamma sarcoglycanopathy, nine limb girdle, one myotonic and one fascioscapulohumeral muscular dystrophy) and five controls were stained with antibody for MHC Class I antigen (Novocastra clone W6/32 HL 1:100 dilution).

RESULTS

Polymyositis and sIBM showed MHC class I antigen positivity along sarcolemma of single and small groups of muscle fibers. The regenerating fibers in the perifascicular area in DM showed intense cytoplasmic positivity of MHC class I antigen. Muscle fibers in all dystrophies except regenerating fibers and control normal muscle were negative for MHC. Capillaries and lymphocytes were positive controls. There were no false positives in the study.

CONCLUSION

MHC Class I immunostaining can be used as a complementary diagnostic tool for the diagnosis of idiopathic inflammatory myopathies.

摘要

目的

研究主要组织相容性复合体(MHC)I类抗原免疫染色在区分特发性炎性肌病和肌营养不良症中的作用。

材料与方法

对40例肌肉活检标本进行MHC I类抗原抗体(诺华卡斯达克隆W6/32 HL,1:100稀释)染色,其中包括7例皮肌炎(DM)、6例多发性肌炎(PM)、2例散发性包涵体肌炎(sIBM)、20例肌营养不良症(1例杜氏肌营养不良症、3例贝克肌营养不良症、4例α型、1例γ型肌聚糖病、9例肢带型、1例强直性和1例面肩肱型肌营养不良症)以及5例对照。

结果

多发性肌炎和散发性包涵体肌炎在单个和小群肌纤维的肌膜处显示MHC I类抗原阳性。皮肌炎束周区域的再生纤维显示MHC I类抗原强烈的胞质阳性。除再生纤维外,所有肌营养不良症的肌纤维以及对照正常肌肉的MHC均为阴性。毛细血管和淋巴细胞为阳性对照。本研究中无假阳性结果。

结论

MHC I类免疫染色可作为诊断特发性炎性肌病的辅助诊断工具。

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