HLA-Ⅱ类基因单倍型与中国北方汉族人皮肌炎/多发性肌炎的相关性研究

目的：探讨HLA-DRB1、DQB1基因单倍型与中国北方地区汉族人皮肌炎，多发性肌炎的相关性。方法：采用聚合酶链反应，序列特异性引物(PCR-SSP)技术，检测中国北方汉族皮肌炎，多发性肌炎患者的HLA-DRB1、DQB1等位基因。结果：与100例正常对照组比较，在52例皮肌炎，多发性肌炎患者中HLA-DRB1^#040x-DQB1^#0301、DRB1^#040x-DQB1^#0401单倍型频率明显增高，经统计学检验两组差别有显著意义，P值分别为0.0307、0.0033。HLA-DRB1^#150x-DQB1^#0602单倍型频率明显降低，P值为0.0201。在38例皮肌炎组中，HLA-DRB1^#040x-DQB1^#0301、DRB1^#040x-DQB1^#0401、DRB1^#070x-DQB1^#0201、DRB1^#120x-DQB1^#0303单倍型频率明显增高，P值分别为0.0292、0.0015、0.0450、0.0192，两组差别有统计学意义。在14例多发性肌炎患者中HLA-DRB1^#070x-DQB1^#0301单倍型频率明显增高，P值为0.0141。结论：特异单倍型可能是决定皮肌炎，多发性肌炎发病及皮肌炎、多发性肌炎异质性的重要因素。     

Recurrent breast cancer at 21 years after resection detected by serum tumor markers and manifested as dermatomyositis

A 52-year-old Japanese woman developed dermatomyositis. She had undergone a standard radical mastectomy for left breast cancer 21 years earlier. Though no physical sign of recurrent breast cancer appeared clinically, levels of tumor markers were abnormally elevated. Therefore, tamoxifen and CAF therapy were given. Further, the clinical course of dermatomyositis almost paralleled the level of serum tumor markers and the clinical course of her recurrent breast cancer. These markers were useful for detecting the recurrence, following the metastatic disease, and monitoring her response to therapy.     

血浆降钙素基因相关肽与多发性肌炎、皮肌炎的关系

目的：本文对血浆降钙素基因相关肽（ＣＧＰＰ）与多发性肌炎／皮肌炎（ＰＭ／ＤＭ） 的关系进行研究,探讨其血管损伤的发病机制。方法：采用放射免疫分析法测定３０例健 康人和３０例ＰＭ／ＤＭ病人ＣＧＲＰ水平变化。结果：ＰＷＤＭ血浆ＣＧＲＰ浓度较正常人降 低（Ｐ＜００１）, ＰＭ／ＤＭ病人有肌酸激酶（ＣＫ）升高和补体Ｃ_３、Ｃ_４下降者其ＣＧＲＰ水平降 低明显（Ｐ＜０.０１）,治疗前后三个月ＣＧＲＰ水平有明显差异（Ｐ＜０．０５）,直线相关分析表 明,血浆ＣＧＲＰ与ＣＫ呈显著负相关,与补体Ｃ_３、Ｃ_４呈显著正相关。结论：ＣＧＲＰ参与了 ＰＭＤＭ血管损伤的发病过程,测定血浆ＣＧＲＰ水平变化可望作为判定病程严重程度和治 疗效果的一个指标,此结果为ＣＧＲＰ治疗ＰＭ／ＤＭ的应用提供依据。     

Automatic decomposition electromyography in idiopathic inflammatory myopathies

Automatic decomposition electromyography (ADEMG) is a commercially available software package with installed reference values that enables the objective measurement of motor unit action potentials (MUAPs). To assess the diagnostic yield of this package in idiopathic inflammatory myopathies (IIM) we performed biceps brachii ADEMG in 17 patients with polymyositis, dermatomyositis and inclusion body myositis. Results were compared with those in 12 controls, and with the results of conventional EMG of the biceps and other muscles. Decreased mean values for MUAP duration occurred significantly more frequently in IIM patients than in controls; other MUAP characteristics did not differ. In IIM patients, decreased mean amplitude and increased mean number of turns occurred significantly less frequently on ADEMG than did corresponding abnormalities on conventional biceps EMG. Decreased mean values for duration and amplitude, and increased mean values for number of turns were seen significantly less often on ADEMG than corresponding abnormalities on conventional EMG of four different, individually chosen muscles. Overall evaluation of ADEMG resulted in a diagnosis of possible myopathy in 1 and probable myopathy in 8 patients, whereas overall evaluation of conventional EMG led to a diagnosis suggestive of IIM in 13 patients. We conclude that, although measurement of mean MUAP duration might be valuable in IIM diagnosis, our results do not favour the use of biceps brachii ADEMG and the installed reference values for the diagnosis of IIM. We suggest modifications to improve ADEMG's applicability.     

以泼尼松为主联合治疗多发性肌炎和皮肌炎32例

目的：比较泼尼松联合疗法和单用治疗多发性肌炎 (PM) 和皮肌炎 (DM)的疗效。方法：PM和DM患者67例分为对照组(单用糖皮质激素)35例，给予泼尼松1 mg·kg 1·d 1，qd。治疗组 32例，给予泼尼松1 mg·kg 1·d 1， qd，加甲氨蝶呤片 7.5～10.0 mg，po，每周1次；加火把花根片3片，tid。疗程4周。主要观察指标有肌力变化，皮疹，血清 ALT、AST、LDH、CK的水平及药物副作用发生率。结果∶治疗后两组患者的肌力均有不同程度改善，肌力改善幅度≥2级患者在对照组中占54.3%，在治疗组中占62.5%，二者差异无显著性(P＞0.05)。两组患者在治疗后血清 ALT、AST、LDH、CK水平均比治疗前有明显下降，差异有极显著性(P＜0.01)。治疗组患者的血清ALT、AST、LDH、CK下降的幅度明显大于对照组，差异有显著性(P＜0.05)。结论：与单用糖皮质激素的疗法相比，联合治疗方法能够使血清肌酶更快地下降，对PM和DM有更好的近期疗效。     

Cutaneous manifestations in neuro-oncology: clinically relevant tumor and treatment associated dermatologic findings

Skin findings are a rare but important aspect of the evaluation and management of patients with tumors of the nervous system. Skin findings have the highest prevalence in genetic tumor syndromes termed neuro-genodermatoses, which include neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and tuberous sclerosis. Skin changes are observed in patients with non-syndromic nervous system malignancy, often as a result of pharmacotherapy. The skin may also manifest findings in paraneoplastic conditions that affect the nervous system, providing an early indication of underlying neoplasm, including dermatomyosistis, neuropathic itch, and brachioradial pruritus. In this article, we review the major cutaneous findings in patients with tumors of the brain, spine, and peripheral nervous system focusing on (1) cutaneous manifestations of genetic and sporadic primary nervous system tumor syndromes, and (2) paraneoplastic neurological syndromes with prominent cutaneous features.     

French expert opinion for the management of juvenile dermatomyositis

A guideline group consisting of a pediatric rheumatologist, internists, rheumatologists, immunologists, a physiotherapist and a patient expert elaborated guidelines related to the management of juvenile dermatomyositis on behalf of the rare autoimmune and autoinflammatory diseases network FAI²R. A systematic search of the literature published between 2000 and 2015 and indexed in PubMed was undertaken. Here, we present the expert opinion for diagnosis and treatment in juvenile dermatomyositis.     

影响多发性肌炎和皮肌炎疗效的因素分析和对策

目的探讨影响多发性肌炎和皮肌炎疗效的因素和对策。方法通过对66例多发性肌炎和皮肌炎的临床资料和治疗方法进行分析。结果延误诊断、糖皮质激素用量不足和疗程不够、不及时加用免疫抑制剂、伴发肺间质病变或心肺功能差及恶性肿瘤是影响其疗效的因素。结论及早诊断,早期足量,尤其早期激素冲击并继之以长时间适当剂量糖皮质激素,及时加用其他免疫抑制剂、尽早发现肺间质疾病与排除恶性肿瘤,可提高疗效,改善预后。