Matsubara S
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Fuchu.
Rinsho Byori. 1995 Sep;43(9):877-85.
The clinical investigation for inflammatory myopathies, which include polymyositis (PM), dermatomyositis (DM) and others, was outlined. The serum creatine kinase (CK) activity increases in the majority of cases of inflammatory myopathies. However, the cases of myositis associated with connective tissue diseases tend to show normal or moderately elevated CK activity. Among the isoenzymes of CK, the MB fraction can increase in the course of treatment as it can originate from regenerating muscle fibres. The macro CK type 1 was reported to appear in association with myositides. Varieties of autoantibodies in the serum such as Jo-1 and Ku have been studied. The Jo-1 antibody is frequently detected in the cases of PM associated with interstitial pulmonary fibrosis. Examination of the heart and lungs is necessary, and so is a search for malignant neoplasms in the cases of DM. Muscle biopsy is mandatory for diagnosing PM, DM and other inflammatory myopathies. Among the latter, inclusion body myositis and granulomatous myopathy need to be identified before treatment as they generally respond poorly. Histological changes of inflammatory myopathies are often distributed unevenly. The magnetic resonance image and ultrasonography are helpful in estimating the distribution of the lesion and therefore in deciding the site of biopsy. Ultrastructural observation of the muscle showed invasion of activated lymphocytes under the basement membrane of the muscle fibres causing degeneration of the myofibrils. The subset analyses of infiltrating cells revealed considerable alterations after the steroid pulse therapy.
概述了对包括多发性肌炎(PM)、皮肌炎(DM)等在内的炎性肌病的临床研究。在大多数炎性肌病病例中,血清肌酸激酶(CK)活性会升高。然而,与结缔组织疾病相关的肌炎病例往往表现为CK活性正常或中度升高。在CK的同工酶中,MB组分可在治疗过程中升高,因为它可能源自再生的肌纤维。据报道,1型巨CK与肌炎有关。已经研究了血清中的多种自身抗体,如Jo-1和Ku。Jo-1抗体在与间质性肺纤维化相关的PM病例中经常被检测到。心脏和肺部检查是必要的,对于DM病例,寻找恶性肿瘤也是必要的。肌肉活检对于诊断PM、DM和其他炎性肌病是必不可少的。在后者中,包涵体肌炎和肉芽肿性肌病在治疗前需要被识别出来,因为它们通常反应不佳。炎性肌病的组织学变化往往分布不均匀。磁共振成像和超声检查有助于评估病变的分布,从而有助于确定活检部位。肌肉的超微结构观察显示,活化淋巴细胞侵入肌纤维的基底膜下,导致肌原纤维变性。浸润细胞的亚群分析显示,在类固醇脉冲治疗后有相当大的变化。