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Nonsarcoid granulomatous myopathy: Two cases and a review of literature

机译:非结节性肉芽肿性肌病:两例并文献复习

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摘要

Granulomatous myopathy is an uncommon skeletal muscles disorder. It can develop in association with other granuloma-forming diseases and is then considered a secondary myopathy or, less frequently, a primary disorder for which no etiology is identified. Studies of granulomatous myopathies have focused on examining the differences between primary and secondary diseases. Herein, we describe two cases of nonsarcoid granulomatous myopathies, for which diagnostic work-up did not reveal an underlying granuloma-causing pathology. The patients exhibited similar histopathological characteristics in skeletal muscle biopsies. However, they had different clinical presentations and therapeutic responses. Specifically, one patient had distal muscle weakness with a poor response to immunosuppressive treatment, whereas the other had a more proximal muscle weakness distribution and a very good response to treatment with corticosteroids and azathioprine, resulting in remission. More studies are warranted to further characterize the clinical course and effect of different treatment modalities on nonsarcoid granulomatous myopathy.
机译:肉芽肿性肌病是一种罕见的骨骼肌疾病。它可以与其他肉芽肿形成疾病一起发展,然后被认为是继发性肌病,或者不常见的原发性疾病,尚无病因。肉芽肿性肌病的研究集中于检查原发性和继发性疾病之间的差异。在这里,我们描述了两个非结节性肉芽肿性肌病的病例,对于这些病例的诊断工作并未发现潜在的肉芽肿性病理。患者在骨骼肌活检中表现出相似的组织病理学特征。但是,他们有不同的临床表现和治疗反应。具体而言,一名患者的远端肌肉无力,对免疫抑制治疗的反应较差,而另一名患者的近端肌肉无力分布更严重,对皮质类固醇和硫唑嘌呤的治疗反应非常好,从而缓解了。有必要进行更多的研究以进一步表征非结节性肉芽肿性肌病的临床过程和不同治疗方式的影响。

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