The physiology of inflammatory myopathies: an overview
The physiology of inflammatory myopathies: an overview
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DOI：
10.1046/j.1365-201x.2001.00822.x
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发表时间：
2001-03-01
期刊：
影响因子：
--
通讯作者：
Lundberg, IE
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文献类型：
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作者：
Lundberg, IE
特发性炎性肌病（IIMs）包括一组起源和发病机制未知的肌肉疾病，其特征在于对称的近端肌无力和肌肉组织中的炎性浸润。肌肉功能丧失背后的机制在很大程度上是未知的。通常认为肌肉无力是由炎症细胞引起的。然而，炎性浸润并不总是存在于肌肉组织中，并且浸润有时具有斑片状分布，这使得仅通过炎性细胞的浸润难以解释全身性肌无力。我们调查了不同阶段的肌炎患者：早期肌炎没有可检测到的炎性浸润，活动性肌炎有明显的炎性浸润和慢性肌炎持续肌无力，但肌肉组织中没有令人愉快的炎性细胞。在这些研究中，观察到临床症状和毛细血管受累与细胞因子白细胞介素（IL）-1 α的表达之间存在更好的相关性，并观察到肌纤维上存在主要组织相容性复合体（MHC）I类表达。这些分子是否会影响肌肉功能尚不清楚。使用磷P-31磁共振波谱法降低三磷酸腺苷（ATP）和磷酸肌酸（PCr）水平的值在休息时观察到。这些代谢异常通过运动进一步加重，PCr水平升高与临床状态改善相关。导致这些生化异常的潜在机制尚未确定，但可能与组织氧合失调有关。
The idiopathic inflammatory myopathies (IIMs) encompass a group of muscle disorders of unknown origin and pathogenesis characterized by symmetrical, proximal muscle weakness and by inflammatory infiltrates in muscle tissue. The mechanisms behind the loss of muscle function are largely unknown. It is often anticipated that the muscle weakness is caused by the inflammatory cells. However, inflammatory infiltrates are not always present in the muscle tissue and the infiltrates sometimes have a patchy distribution, which makes it difficult to explain the generalized muscle weakness merely by infiltration of inflammatory cells. We investigated patients at different stages of myositis: early myositis without detectable inflammatory infiltrates, active myositis with pronounced inflammatory infiltrates and chronic myositis with persisting muscle weakness but without delectable inflammatory cells in muscle tissues. In these studies, a better correlation was observed between the clinical symptoms and involvement of the capillaries with expression of the cytokine interleukin (IL)-1 alpha and by the presence of major histocompatibility complex (MHC) class I expression on muscle fibres. Whether these molecules could affect muscle function is not known. Using phosphorus P-31 magnetic resonance spectroscopy decreased values of adenosine triphosphate (ATP) and phosphocreatine (PCr) levels were observed at rest. These metabolic abnormalities were further accentuated by exercise and increased PCr levels correlated with improved clinical status. The underlying mechanisms responsible for these biochemical abnormalities have not been defined but could be related to a disturbed tissue oxygenation.