Dimitri Dalia, Dubourg Odile, Maisonobe Thierry, Fournier Emmanuel, Ranque Brigitte, Laforêt Pascal, Mussini Jean-Marie, Pagnoux Christian, Béhin Anthony, Papo Thomas, Benveniste Olivier, Eymard Bruno, Herson Serge
Centre de Référence des Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye, APHP, INSERM U841, CHU Henri Mondor, 51 boulevard du Maréchal de Lattre de Tassigny, 94010 Créteil, France.
Neuromuscul Disord. 2008 Jun;18(6):493-500. doi: 10.1016/j.nmd.2008.04.015. Epub 2008 Jun 4.
New classification of idiopathic inflammatory myopathy (IIM) defined three major entities, polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (s-IBM). We report the clinical, electrophysiological and pathological characteristics of three patients with a rare form of IIM not fulfilling the diagnostic criteria for any of these three major entities. The three patients presented with a subacute, distal asymmetrical weakness in upper limbs. Muscle biopsy showed an active myositis, with necrosis and regeneration, T cell infiltrates with invasion of non-necrotic fibers, without rimmed vacuoles, and diffuse major histocompatibility complex-I (MHC-I) immunostaining in muscle fibers. All patients responded to immunosuppressive agents. Seven others cases were identified in the literature. It is important to recognize this atypical presentation as it seems to respond to immunosuppressive agents.
特发性炎性肌病（IIM）的新分类定义了三个主要类型，即多发性肌炎（PM）、皮肌炎（DM）和散发性包涵体肌炎（s-IBM）。我们报告了三例罕见形式的IIM患者的临床、电生理和病理特征，这三例患者均不符合上述三种主要类型的诊断标准。这三名患者均表现为亚急性、上肢远端不对称性肌无力。肌肉活检显示为活动性肌炎，伴有坏死和再生，T细胞浸润并侵入非坏死纤维，无镶边空泡，且肌纤维中主要组织相容性复合体-I（MHC-I）弥漫性免疫染色。所有患者对免疫抑制剂均有反应。文献中还发现了其他七例病例。认识到这种非典型表现很重要，因为它似乎对免疫抑制剂有反应。
