Clinicopathological features of sarcoidosis manifesting as generalized chronic myopathy
Clinicopathological features of sarcoidosis manifesting as generalized chronic myopathy
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DOI：
10.1007/s00415-015-7680-0
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发表时间：
2015-04-01
影响因子：
6
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Maeshima, Shinya;Koike, Haruki;Sobue, Gen
虽然慢性肌病已报道影响骨骼肌结节病，其临床病理特征尚未完全阐明。我们描述了11例结节病患者的临床、组织病理学和预后特征，这些结节病表现为慢性进行性、全身性肌病，并经肌肉活检证实。全身性肌无力延伸到四肢和躯干是这些病例的主要特征。9例患者明显出现肌肉萎缩，尤其是下肢，而仅有2例患者出现肌痛。5例结节病以肌病为首发表现。只有6例患者出现血浆肌酸激酶水平升高。骨骼肌计算机断层扫描显示，肌肉萎缩主要分布在髋关节内收肌、膝关节屈肌和踝关节跖屈肌。放射学评估，包括磁共振成像、镓闪烁成像和氟脱氧葡萄糖正电子发射断层扫描-计算机断层扫描成像，显示只有一半的检查患者有骨骼肌炎症。然而，在所有患者中，肌肉活检标本显示活跃的炎症过程，如局灶性非干酪化上皮样肉芽肿，以cd4阳性淋巴细胞浸润为主。肌肉瘤经HLA-ABC、HLA-DR和细胞间黏附分子-1抗体弥漫性染色，提示弥漫性和活性抗原呈递。病程短的患者经免疫调节治疗后功能改善明显更好（p < 0.05）。病程较长的患者治疗效果较差；因此，早期诊断和早期治疗非常重要。
Although chronic myopathy has been reported to affect skeletal muscle in sarcoidosis, its clinicopathological features have not been fully elucidated. We characterized the clinical, histopathological, and prognostic features of eleven patients with sarcoidosis manifesting with chronically progressive, generalized myopathy that was confirmed with muscle biopsy. Generalized muscle weakness extending to the four extremities and trunk was the cardinal feature of these cases. Muscle atrophy was evident in nine patients, particularly in the lower limbs, whereas myalgia was reported in only two patients. Myopathy was the first manifestation of sarcoidosis in five patients. Only six patients showed elevated plasma creatine kinase levels. Using skeletal muscle computed tomography, the distribution of muscle atrophy was predominant in the hip adductors, knee flexors and ankle plantarflexors. Radiological assessments, including magnetic resonance imaging, gallium scintigraphy, and fluorodeoxyglucose positron emission tomography-computed tomography imaging, revealed findings suggestive of skeletal muscle inflammation in only half of the patients examined. However, in all patients, muscle biopsy specimens revealed an active inflammatory process, as observed by focal non-caseating epithelioid granuloma with predominant CD4-positive lymphocytic infiltration. Sarcolemmas were diffusely stained with HLA-ABC, HLA-DR and intercellular adhesion molecule-1 antibodies, suggesting diffuse and active antigen presentation. Functional improvement after immunomodulatory treatment was significantly better in patients with short disease durations (p < 0.05). The therapeutic response was poor in patients with long disease durations; thus, early diagnosis and early initiation of treatment are important.