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[从局限性结节性肌炎连续发展为嗜酸性粒细胞性肌炎的病例]

[Case of eosinophilic myositis in continuum from localized nodular myositis].

作者信息

Matsuse Dai, Ikezoe Koji, Shigeto Hiroshi, Murai Hiroyuki, Ohyagi Yasumasa, Kira Jun-ichi

机构信息

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.

出版信息

Rinsho Shinkeigaku. 2008 Jan;48(1):36-42. doi: 10.5692/clinicalneurol.48.36.

Abstract

We report a 72-year-old man with eosinophilic myositis (EM). At age 71 he noticed a painful nodule in his left calf. A biopsy (first biopsy) showed marked infiltration of mononucleated cells and necrotic muscle fibers. Several phagocytosed fibers were also seen. He was diagnosed as having myositis. The painful nodule disappeared spontaneously. At age 72, he again had a painful nodule, but this time in his right calf; again, this disappeared spontaneously on the first admission. Just after discharge, he noted painful nodules in the left thigh and right anterior tibial muscles and was again admitted (second admission). Neurological examination revealed mild proximal-dominant weakness in all four extremities but no other abnormalities. Laboratory studies showed elevated creatine kinase (CK) level (38,803 U/l; normal 62-287) and positive Jo-1 antibody, but no eosinophilia. Needle electromyography of the limb muscles showed myogenic patterns. Magnetic resonance imaging of the lower limbs demonstrated several T2-high and gadolinium (Gd)-enhanced lesions. Muscle biopsy (second biopsy) from the left quadriceps femoris showed marked infiltration of eosinophils; he was diagnosed as having EM. Administration of prednisolone was initiated at 60 mg/day and then gradually tapered. After starting treatment with steroids, his muscle weakness gradually ameliorated, CK level dramatically decreased, and the nodules disappeared. Clinically, the patient had developed localized nodular myositis (LNM), but pathologically it was EM without peripheral blood eosinophilia and positive Jo-1 antibody that is occasionally found in polymyositis (PM). Thus, this patient demonstrated overlapping characteristics of EM, LNM, and possibly PM, suggesting that a common mechanism underlay these conditions. As discussed, the involvement of eosinophils in three inflammatory myopathies was indicated.

摘要

我们报告了一名72岁的嗜酸性粒细胞性肌炎(EM)男性患者。71岁时,他注意到左小腿有一个疼痛性结节。一次活检(首次活检)显示单核细胞和坏死肌纤维显著浸润。还可见一些被吞噬的纤维。他被诊断为患有肌炎。疼痛性结节自行消失。72岁时,他再次出现一个疼痛性结节,但这次在右小腿;同样,在首次入院时该结节又自行消失。出院后不久,他注意到左大腿和右胫前肌出现疼痛性结节,于是再次入院(第二次入院)。神经学检查显示四肢均有轻度以近端为主的肌无力,但无其他异常。实验室检查显示肌酸激酶(CK)水平升高(38,803 U/l;正常范围62 - 287),Jo - 1抗体阳性,但无嗜酸性粒细胞增多。肢体肌肉的针极肌电图显示为肌源性模式。下肢磁共振成像显示多个T2高信号和钆(Gd)强化病变。左股四头肌的肌肉活检(第二次活检)显示嗜酸性粒细胞显著浸润;他被诊断为患有EM。开始给予泼尼松龙60 mg/天,然后逐渐减量。开始使用类固醇治疗后,他的肌无力逐渐改善,CK水平显著下降,结节消失。临床上,该患者发展为局限性结节性肌炎(LNM),但病理上为EM,无外周血嗜酸性粒细胞增多且有在多发性肌炎(PM)中偶尔发现的Jo - 1抗体阳性。因此,该患者表现出EM、LNM以及可能的PM的重叠特征,提示这些情况存在共同机制。如前所述,嗜酸性粒细胞参与了三种炎症性肌病。

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