MRI在多发性肌炎/皮肌炎诊断中的价值

目的:探讨MRI在多发性肌炎(PM)/皮肌炎(DM)诊断中的应用价值。方法:回顾分析临床资料完整的24例PM/DM患者的肌肉及皮下软组织MR图像,其中PM 16例,DM 8例。检查序列包括FSE T1WI、FSE T2WI、fs T2WI。扫描双侧大腿,常规冠状位、矢状位及横轴位平扫。结果:24例PM/DM中19例大腿肌肉MRI上存在异常,表现为:肌肉炎性水肿16例,肌筋膜炎10例,皮下软组织水肿6例,2例肌肉萎缩。结论:MRI在PM/DM诊断中有重要应用价值,肌肉和皮下组织炎性水肿是PM/DM患者最常见的MRI表现。MRI fs T2WI序列能更好地检出炎性病变。     

A statistical matching of patients with dermatomyositis and polymyositis: A preliminary feasibility study

A mathematical system of matching disease severity in groups of patients with dermatomyositis and polymyositis is described. The method consists of assigning scores to the different clinical and laboratory features, the maximum of those scores arbitrarily being the ‘gold standard’. By this method it is possible to compare different groups of patients in terms both of their overall disease and of muscle involvement. The method may prove of value in meta-analysis and in the reporting of studies in these diseases. Efforts are now being made to reach international agreement on the scoring system. ‘To sleep: perchance to dream’ William Shakespeare,Hamlet ‘To diagnose: perchance to match’Ipse dixits     

多发性肌炎/皮肌炎83例临床及病理分析

目的探讨多发性肌炎/皮肌炎(PM/DM)的临床及病理特点,以及这些特点与疗效及预后的关系。方法对83例PM/DM患者的临床资料进行总结、分析。结果PM/DM以肌无力、肌肉压痛、血清肌酶谱增高为主要临床表现,伴肌电图及病理学检查异常;经肾上腺皮质激素和免疫抑制剂等治疗后,总有效率86.7 %。结论PM/DM各型之间临床与病理表现的差异提示可能存在不同的发病机制。神经损害是全身系统损害的一部分。PM/DM的预后与分型、治疗时机有关,早期强有力的治疗可望改善预后。     

豚鼠多发性肌炎动物模型的肌电图研究

以兔肌匀浆加完全弗氏医剂免疫豚鼠制作实验性多发性肌炎动物模型,实验前与实验后2、4、6、8周分别检测肌电图及坐骨神经运动传导潜速率。首次免疫注射4周后肌电图开始出现异常,6及8周大多数表现为多相波增多、时限缩短、波幅降低。肌电图改变与肌肉病理及肌酶谱改变同步,提示肌电图改变是确定实验性肌炎的标准之一。     

Magnetic resonance imaging of the muscles in patients with polymyositis and dermatomyositis

Magnetic resonance imaging (MRI) of the muscles was performed in patients with polymyositis and dermatomyositis. Lesions with high intensity on T2-weighted image, but normal intensity on T1-weighted image, were observed in 7 of 8 patients in the active stage of the disease. Following clinical improvement with corticosteroid therapy in 4 patients, the high intensity lesions reverted to normal. The high intensity lesions seen on T2-weighted image in the active stage may represent edema and inflammation of the muscle. MRI of the muscle may serve as a diagnostic tool and be useful for follow-up of the patients with polymyositis or dermatomyositis.     

Treatment of inflammatory myopathies

Introduction: The idiopathic inflammatory myopathies (IIM) dermatomyositis (DM) and polymyositis (PM) are chronic diseases affecting the striated muscles with variable involvement of other organs.

Glucocorticoids are considered the cornerstone of treatment, but some patients require adjunctive immunosuppressive agents because of insufficient response to glucocorticoids, flares upon glucocorticoid tapering, or glucocorticoid-related adverse events.

Areas covered: The aim of this article was to review (PubMed search until February 2018) the evidence on established and new therapies derived from randomized controlled trials (RCTs) on adult DM and PM. In addition, key data from open-label trials, case reports, and abstracts were included where data from RCT were lacking.

Expert commentary: Numerous synthetic and biological immunosuppressive agents are currently available to treat the IIM, sometimes in combination. The choice of the specific medication in the individual patient depends upon the disease phenotype and patient’s characteristics. Exercise improves muscle performance without causing disease flares and should be an integral part of the treatment of the IIM. Prompt diagnosis and treatment can lead to better outcome.     

Muscle metabolism in patients with polymyositis simultaneously evaluated by using 31P-magnetic resonance spectroscopy and near-infrared spectroscopy

Simultaneous measurements of muscle energy metabolism using (31)P-magnetic resonance spectroscopy ((31)P-MRS) and the kinetics of muscular oxygen metabolism using near-infrared spectroscopy (NIRS) were conducted in polymyositis (PM) patients. The subjects were 12 PM patients (age 45 +/- 12 years) and 12 normal controls (age 41 +/- 12 years). The muscle phosphocreatine (PCr) index and intracellular pH (pHi) were determined with (31)P-MRS and the changes in intramuscular oxygenated (oxy-Hb), deoxygenated (deoxy-Hb), and total haemoglobin (total Hb) were evaluated with NIRS . The pHi and PCr index before steroid therapy in PM patients were significantly lower during exercise than in normal controls, and their recovery was statistically significantly delayed compared with the controls. The pattern of changes in NIRS over time before steroid therapy in PM patients differed from that in normal controls. There were smaller changes in deoxy-Hb and oxy-Hb during exercise, and total Hb decreased during exercise. In contrast, the kinetics of muscular metabolism after steroid therapy showed changes similar to those seen in normal controls. Simultaneous (31)P-MRS and NIRS measurements to determine the kinetics of muscular metabolism are expected to be useful as a noninvasive approach for the evaluation of treatment effects in PM patients.     

多发性肌炎/皮肌炎患者血清中外泌体的分离鉴定

目的从多发性肌炎/皮肌炎(polymyositis/dermatomyositis,PM/DM)患者外周血血清中分离鉴定外泌体(exosome),并进行初步的蛋白质研究。方法用Exo QuickTM试剂盒分离纯化PM/DM患者血清exosome;透射电镜观察其形态特征,Nanosight可视型纳米颗粒分析仪检测粒径大小分布情况;western blot鉴定exosome的表面标志物CD9,CD81,Flotillin-2;BCA法对其所携带的蛋白质进行定量分析;十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE)初步分析其蛋白质组分。结果透射电镜下患者血清exosome呈圆形或椭圆形膜性小囊泡,直径分布范围92±67 nm;western blot结果表明,PM/DM血清来源的exosome存在表面标志物CD9、CD81、Flotillin-2;BCA法测定PM/DM患者exosome总蛋白浓度为14.68(6.00,32.55)μg/μL,健康组exosome总蛋白浓度为14.09(8.00,23.28)μg/μL,SDS-PAGE显示两组血清exosome均在Mr(×103)为55~70处有高丰度蛋白质富集,在Mr(×10~3)为40~55处存在差异条带。结论成功从PM/DM患者的外周血中分离出血清exosome,并为寻找差异蛋白质提供实验依据。     

83例多发性肌炎/皮肌炎伴呼吸系统损害的观察与护理

目的探讨多发性肌炎/皮肌炎伴呼吸系统损害患者的临床护理策略。方法选择83例多发性肌炎/皮肌炎伴呼吸系统损害患者为研究对象,护理人员在治疗期间实施针对性护理方法,观察护理效果。结果 83例患者中,除1例发生呼吸衰竭死亡,3例自主放弃治疗外,79例患者病情得到有效控制,好转出院,总有效率95.18%。结论多发性肌炎/皮肌炎伴呼吸系统损害患者治疗期间,护理人员根据具体症状进行原因分析,实施针对性护理措施,可提高提高疗效,改善疾病转归。