Department of Pathology and Molecular Medicine, Charles University, 2nd Medical Faculty and University Hospital Motol, Prague, Czech Republic.
1st Department of Infectious Diseases, Charles University, 2nd Medical Faculty and University Hospital Bulovka, Czech Republic.
J Cell Mol Med. 2009 Aug;13(8B):2008-2018. doi: 10.1111/j.1582-4934.2008.00431.x. Epub 2008 Jul 24.
Selective atrophy of type II muscle fibres has been long recognized as an enigmatic but consistent feature of myasthenia gravis (MG) muscle; however, the pathophysiology and the mechanism of that change have remained obscure. In the present study, the results of histomorphometric analysis performed on muscle biopsies from 207 thymectomized seropositive MG patients were correlated with clinical features of MG to demonstrate possible pathophysiological associations and potential prognostic impact. The atrophy of type II fibres was verified in 35 cases (16.9%), being more pronounced in fibres of IIB subtype. It was neither significantly associated with the duration and severity of MG nor with the age of the patients. On the other hand, we demonstrated that the atrophy associated with long-term treatment with corticosteroids, and correlated with increasing doses. Thus, we suppose that the atrophy of type II muscle fibres in seropositive MG is steroid induced rather than MG-associated event. Although the MG patients with atrophy of type II fibres did not differ from the remaining MG cases in terms of improvement in the disease during the follow-up period, our analysis provides clear evidence that they presented a significantly slower tendency to reach an asymptomatic state after thymectomy. Therefore, the steroid-induced atrophy of type II fibres in MG muscle might be considered to be an unfavourable prognostic factor.
II 型肌纤维选择性萎缩一直被认为是重症肌无力(MG)肌肉的一个神秘但一致的特征;然而,其变化的病理生理学和机制仍不清楚。在本研究中,对 207 例胸腺瘤阳性 MG 患者的肌肉活检进行组织形态计量学分析的结果与 MG 的临床特征相关联,以证明可能的病理生理学关联和潜在的预后影响。在 35 例(16.9%)中验证了 II 型纤维的萎缩,在 IIB 亚型的纤维中更为明显。它与 MG 的持续时间和严重程度或患者的年龄均无显著相关性。另一方面,我们证明与长期皮质类固醇治疗相关的萎缩与剂量增加相关。因此,我们假设胸腺瘤阳性 MG 中的 II 型肌纤维萎缩是类固醇诱导的,而不是与 MG 相关的事件。尽管在随访期间,II 型纤维萎缩的 MG 患者在疾病改善方面与其余 MG 病例没有差异,但我们的分析提供了明确的证据表明,他们在胸腺切除术后达到无症状状态的趋势明显较慢。因此,MG 肌肉中类固醇诱导的 II 型纤维萎缩可能被认为是一个不利的预后因素。