Polymyositis in a patient with recurring ovarian cancer and history of unrelated breast cancer

Background Polymyositis (PM) is an idiopathic inflammatory myopathy. Occasionally, it may present as a paraneoplastic syndrome and it is strongly associated with ovarian and weakly with breast cancer. We present here a case of a 60-year-old patient with sequential breast and ovarian (second primary) carcinomas followed by PM as a paraneoplastic disorder. Case report The patient had been diagnosed with stage I breast carcinoma 3 years ago and had been treated with conservative surgery followed by radiotherapy and six cycles of chemotherapy (CMF). One and a half year later an ovarian carcinoma was diagnosed for which the patient underwent abdominal hysterectomy oophorectomy and omentectomy. The pathological report characterized it as second primary. Adjuvant chemotherapy with carboplatin and taxol was administered. Fourteen months after the initial laparotomy, the patient was re-operated due to ovarian carcinoma recurrence which was involving all lesser pelvis organs. After a successful radical removal of the recurrence the patient developed a fully expressed PM. This case serves to remind that this disease can occur as a paraneoplastic disorder.     

Bilateral leg pain and unilateral calf atrophy caused by polymyositis accompanying lumbar spinal stenosis and disc herniation: a case report

Polymyositis is a subgroup of idiopathic inflammatory myopathies characterized by symmetric proximal limb weakness and chronic skeletal muscle inflammation. We herein report the first case of bilateral leg pain and unilateral calf atrophy caused by polymyositis accompanying lumbar spinal stenosis and disc herniation. A 52-year-old man presented with intermittent claudication and calf pain that had become gradually aggravated during the last 3 months. Magnetic resonance imaging showed spinal stenosis at the L3/4 and L4/5 levels and lumbar disc herniation at the L4/5 level. Preoperative laboratory investigations revealed elevated muscle enzyme concentrations. Magnetic resonance imaging also showed atrophy, fatty degeneration, and edema in both calf muscles. Histological examination showed inflammatory myositis and fibrosis in the perifascicular connective tissues. The patient was diagnosed with polymyositis. We performed decompressive laminectomy at the L3/4 and L4/5 levels and discectomy at the L4/5 level. After administration of prednisolone for 6 months and methotrexate for 3 months, the patient’s bilateral calf pain and abnormal laboratory findings improved. The combination of surgical decompression and adequate medical treatment resulted in a successful recovery. Polymyositis should be suspected in patients with lumbar spinal stenosis or lumbar disc herniation who exhibit increased muscle enzyme concentrations or lower extremity muscle atrophy.     

多发性肌炎/皮肌炎治疗研究进展

多发性肌炎/皮肌炎(polymyositis,PM/dermatomyositis,DM)是以侵犯骨骼肌为主,可累及肌肉、肺、肾等多个脏器的自身免疫性疾病,其临床表现复杂,患者多因肺间质病变、肿瘤等并发症死亡,治疗以糖皮质激素为主,以及免疫抑制剂、生物制剂等,但不良反应大,对相关并发症的效果不理想。而干细胞移植治疗是作为当前治疗难治性PM/DM的一种手段。本文从PM/DM的治疗现状出发进行综述,以期为PM/DM临床治疗提供更多参考。     

重叠综合征并发横纹肌溶解症一例

74岁女性患者,手足及口周部位皮肤变硬2个月余,伴乏力、酱油色尿1个月。血清肌酸激酶4 242 U/L,肌红蛋白1 124 ng/ml。诊断为重叠综合征并发横纹肌溶解症。经甲泼尼龙80 mg每日1次、甲氨蝶呤15 mg每周1次治疗,效果不理想,转入肾病内科行血液净化治疗。血清肌红蛋白恢复正常后,继续口服甲泼尼龙40 mg每日1次,定期复诊并缓慢减量。目前甲泼尼龙减量至16 mg/d,己持续治疗1年,血清肌酸激酶及肌红蛋白均在正常范围。     

磁共振成像在多发性肌炎和皮肌炎治疗评价中的初探

目的 探讨多发性肌炎和皮肌炎治疗前后磁共振成像( MRI)检查的价值.方法 纳入15例多发性肌炎及皮肌炎患者,治疗前后分别行患者双下肢近端肌群MRI检查,检测血清肌酸激酶水平,并测定肌力;对比治疗前后肌力与MRI结果之间、肌力与肌酸激酶之间的相关性.统计学处理采用独立样本t检验及Pearson相关分析.结果 治疗前患者MR[计分2.37±0.62,肌酸激酶(3841±3175) U/L,肌力评分15.1±2.4;治疗后MRI计分1.30±0.28,肌酸激酶(549±338) U/L,肌力评分18.1±0.9,差异有统计学意义(P＜0.05).肌力与MRI计分、肌酸激酶水平均有相关性,且与前者呈强相关(r=-0.890,P＜0.05).结论 MRI能更好地反映多发性肌炎及皮肌炎患者肌力改善情况,较血清肌酸激酶有更高的敏感性.     

慢性移植物抗宿主病相关性多发性肌炎一例临床和骨骼肌病理特点

目的 报道1例慢性移植物抗宿主病相关性多发性肌炎患者的临床和骨骼肌病理改变特点.方法 我院于2010年12月29日收治1例慢性移植物抗宿主病相关性多发性肌炎患者,该患者为女性,40岁,因急性粒单核细胞白血病于20个月前行异基因造血干细胞移植术.术后给予环孢素A抗排异治疗13个月,6个月前缓慢出现进行性四肢近端无力,伴有肌痛.血肌酸激酶升高(426～1948 U/L).血清抗EJ抗体强阳性.肌电图提示神经源性损害,周围神经传导速度下降.对该患者进行左肱二头肌活体组织检查,标本除进行组织学、酶组织化学染色外,还采用抗CD8、CD20、CD68和主要组织相容性复合物-Ⅰ (MHC-Ⅰ)鼠抗人单克隆抗体作为第一抗体进行免疫组织化学染色.结果 骨骼肌的主要病理改变是肌纤维直径变异加大,伴随肌纤维坏死、再生以及成小组分布的角状萎缩肌纤维.血管周围和肌内衣可见呈灶性分布的CD8+T淋巴细胞和CD6+8单核细胞浸润.MHC-Ⅰ染色显示大部分肌纤维膜异常着色.结论 慢性移植物抗宿主病相关性多发性肌炎具有慢性炎性肌肉病特点,可以伴随神经源性骨骼肌损害.     

神经肌炎与多发性肌炎的对比分析

目的 :为了认识神经肌炎的临床、神经电生理等方面特点 ,探讨其与多发性肌炎的关系。方法 :选取我院近 10年间收治的较典型的 11例神经肌炎病人 ,随机选取同期收治的多发性肌炎病人 2 9例 ,对此两组病人的临床表现、心脏并发症、对激素治疗的疗效、肌电图以及肌肉活检的结果给予对比研究。结果 :在神经肌炎组中 ,有 8例感觉障碍 (6例呈末梢型感觉障碍分布 ;2例呈多发性神经根神经炎表现 ) ;神经传导速度检查中 ,10例有运动传导速度明显减慢或引不出 ,1例电位波幅明显降低 ,呈轴索样损害的表现 ;多发性肌炎组病人均无感觉障碍 ,神经传导速度检查均正常。两组在其他临床表现及激素治疗效果上 ,血清CK增高 ,肌活检、肌电图方面无明显差异(P >0 0 5 )。结论 :神经肌炎是多发性肌炎中一种少见的特殊类型 ,除具有多发性肌炎的表现之外 ,同时还具有周围神经病的表现 ,两者间是很难区分的     

肌纤维内脂滴增多在炎性肌病中的意义

目的　了解在炎性肌病中肌纤维内脂滴增多的发生频率、影响因素及临床意义。方法回顾性分析53例多发性肌炎和皮肌炎患者的临床病理资料,通过油红O染色显示肌纤维脂滴,依据肌纤维内脂滴的含量将患者分为大致正常和明显增多两组,比较各组患者肌力、肌酶谱、肌肉组织病变程度、病程和用皮质类固醇治疗情况。结果　在53例肌肉标本中, 37 7%有脂滴明显增多。与脂滴正常组相比,脂滴明显增多组肌纤维坏变程度和临床肌力下降程度差异有统计学意义。虽然用激素治疗患者出现脂滴明显增多的几率增加,但无统计学意义。结论　部分肌炎患者有肌纤维内脂滴增多,主要与肌纤维病变有关,细胞能量代谢的异常又可能促进肌纤维的病变,加重肌肉无力。     

甲状腺功能减退性肌病误诊为多发性肌炎10例分析

目的探讨甲状腺功能减退性肌病与多发性肌炎容易误诊的原因.方法回顾分析10例甲状腺功能减退性肌病患者的临床表现及实验室检查资料,并复习有关文献.结果甲状腺功能减退性肌病与多发性肌炎在肌肉方面的表现相似,但甲状腺功能检查可以鉴别,左甲状腺素替代治疗有效.结论对于肌肉无力和肌酶升高者需常规检测甲状腺功能.     

误诊为多发性肌炎的脂质沉积性肌病——附3例报告

目的探讨脂质沉积性肌病(lipid storage myopathy,LSM)的临床特征、病理改变、代谢异常及治疗。方法回顾性分析我院确诊的3例LSM患者的临床表现、肌肉病理所见及血、尿代谢检查结果 ,结合文献报道总结LSM的临床特点和发病机制,并对多发性肌炎的诊断标准进行讨论。结果 3例患者均有对称性肌无力和/或肌痛,肌酶谱增高,最初均诊断为多发性肌炎,经激素治疗可好转,但病情反复。肌肉病理显示肌纤维大小不等,大量肌纤维内可见类圆形空泡,油红O染色见肌纤维内大量红染的脂滴沉积。经调整饮食、口服维生素B2、左卡尼汀等药物治疗后,症状改善明显。结论应用Bohan/Peter标准可能导致对多发性肌炎的过度诊断。对临床表现为肌无力、肌痛的患者,不要盲目应用激素治疗,应积极进行骨骼肌活检,明确诊断。