恶性肿瘤相关性皮肌炎/多发性肌炎

皮肌炎/多发性肌炎是一种原因不明的炎性肌肉疾病,常伴发恶性肿瘤,常见的为卵巢癌、乳腺癌、肺癌、胃肠道肿瘤等,而鼻咽癌在亚洲国家较常见.恶性肿瘤相关皮肌炎/多发性肌炎可能的高危因素包括高龄、男性患者、血沉增高、严重的皮损等,其发病机制可能为肿瘤细胞与肌细胞之间相似抗原的交叉免疫反应等.血清自身抗-155抗体是癌症相关性皮肌炎/多发性肌炎的血清学监测指标.     

皮肌炎／多发性肌炎患者血清Jo—1抗体检测的临床意义

应用免疫印迹技术测定５２例皮肌炎和多发性肌炎病人血清可提取性核抗原（ＥＮＡ）多肽抗体，其中Ｊｏ－１抗体的阳性率分别为１３．３％和３１．８％。１１例Ｊｏ－１抗体阳性病人中７２．７％起较急对类固醇治疗反应差，减药或停药易于复；４５．５％伴发间质性肺病，５４．５％伴有Ｒａｙｎａｎｄ现象，６３．６％合并关节炎，３６．４％合并恶性肿瘤。４１例Ｊｏ－１抗体阴性病人上述合并症阳性率仅分别为９．８％、１７．１％、     

多发性肌炎/皮肌炎患者肠道病毒核糖核酸和中和抗体的检测及其意义

目的 探讨肠道病毒感染在多发性肌炎／皮肌炎（ＰＭ／ＤＭ）发病机理中的作用和意义。方法 用逆转录聚合酶链反应（ＲＴ／ＰＣＲ）和中和试验分别检测１６例ＰＭ／ＤＭ患者和１０例正常人对照组外周血中肠道病毒核糖核酸（ＲＮＡ）和抗体滴度。结果 １６例ＰＭ／ＤＭ患者中肠道病毒糖核酸检出率为５０。．０％,病毒中和试验中和抗体阳性率１２．５％。     

抗Jo-1抗体与皮肌炎和多发性肌炎临床关系的探讨

目的探讨抗Jo-1抗体与皮肌炎和多发性肌炎（PM／DM）的临床关系。方法分析16例抗Jo-1抗体阳性的多发性肌炎／皮肌炎（PM／DM）的临床特征,并与81例阴性组进行比较。结果本组中抗Jo-1抗体阳性占16．5％,抗Jo-1抗体阳性组出现肺间质病变（ILD）11例（68．75％）、肺部感染15例（93．75％）、和多关节炎／关节痛8例（50．00％）、肌肉活检异常3例（18．75％）、肌力异常4例（25．00％）、RF阳性5例（55．50％）、技工手4例（25．00％）、发热14例（87．50％）、皮肤硬肿5例（31．25％）与抗Jo-1抗体阴性组比较,差异显著（P〈0．05）。结论在多发性肌炎／皮肌炎（PM／DM）中,抗Jo—1抗体阳性者有明显的肺间质病变、肺部感染、多关节炎、RF阳性、技工手、发热、皮肤硬肿等临床特征,提高对Jo-1抗体综合征的认识,早期诊断,早期治疗,提高生存率。     

特发性炎症性肌病患者血清铁蛋白的检测及临床意义

目的 探讨皮肌炎、临床无肌病性皮肌炎和多发性肌炎等特发性炎症性肌病患者的血清铁蛋白水平与疾病活动性特别是肺间质病变之间的关系。 方法 分析120例特发性炎症性肌病患者铁蛋白与炎症指标、自身抗体、血清肌酶以及肺间质病变严重程度的相关性。 结果 ①36例患者铁蛋白升高,84例铁蛋白正常。铁蛋白升高组的C反应蛋白（14.1 ± 6.5） mg/L、血细胞沉降率（34.8 ± 8.2） mm/1 h、天冬氨酸转氨酶（111.8 ± 44.6） U/L和乳酸脱氢酶（388.6 ± 81.5） U/L分别高于铁蛋白正常组的C反应蛋白（3.6 ± 1.7） mg/L、血细胞沉降率（15.4 ± 2.7） mm/1 h、天冬氨酸转氨酶（46.0 ± 9.0） U/L、乳酸脱氢酶（260.7 ± 29.1） U/L ;均P < 0.01。但两组之间肌酸激酶水平差异无统计学意义;②合并急进性/亚急性肺间质病变和慢性肺间质病变患者的铁蛋白水平分别为（650.5 ± 268.5）和（489.9 ± 157.3） ng/ml,两者均显著高于不伴有肺间质病变（155.7 ± 90.8） ng/ml以及仅有影像学改变而无临床症状的肺间质病变患者（193.3 ± 62.1） ng/ml,均P < 0.01;③铁蛋白升高组慢性肺间质病变（52.8%）和急进性/亚急性肺间质病变（22.2%）的发生率高于正常组（慢性肺间质病变为25.9%,急进性/亚急性肺间质病变为3.5%）,均P < 0.01;④铁蛋白升高组肺高分辨率CT的磨玻璃样影（67.6%）和蜂窝状改变（14.7%）的发生率分别高于正常组（磨玻璃样影43.4%,蜂窝状2.6%, 均P < 0.05）。 结论 在特发性炎症性肌病中,铁蛋白与血细胞沉降率、C反应蛋白、天冬氨酸转氨酶、乳酸脱氢酶、肺间质病变的发生以及肺高分辨率CT的影像学之间高度相关,提示其在判断疾病活动性特别是肺间质病变病情严重程度中有重要的临床意义。     

血清Jo—1抗体与皮肌炎／多发性肌炎的临床意义探讨

对35例皮肌炎/多发性肌炎（DM/PM）患作回顾性分析,比较抗Jo-1抗体阳性的DM/PM患临床特点的差异,结果发现抗Jo-1抗体阳性间质性肺病,关节炎,恶性肿瘤和Raynaud现象的伴发率均明显高于抗Jo-1抗体阴性,且起病较急,病情进展快,需要大量的皮质类固醇激素才能控制病情,提示抗Jo-1抗体阳性的DM/PM患病情重,预后差,临床治疗应积极,及时,皮质类固醇激素的维持时间应足够长。     

皮肌炎自身抗体检测研究进展

一些自身抗体包括抗黑素瘤分化相关基因5抗体、抗转录中介因子1γ抗体、抗核基质蛋白2抗体、抗甘氨酰-tRNA合成酶抗体、抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体、抗组氨酰-tRNA合成酶抗体等与皮肌炎的临床表型和预后密切相关.抗黑素瘤分化相关基因5抗体、抗甘氨酰-tRNA合成酶抗体、抗组氨酰-tRNA合成酶抗体与皮肌炎并发间质性肺病有关,特别是抗黑素瘤分化相关基因5抗体与皮肌炎并发的间质性肺病的发生、病情活动和患者高死亡率显著相关.通常抗黑素瘤分化相关基因5抗体阳性的患者对常规的免疫抑制治疗疗效欠佳.抗转录中介因子1γ抗体、抗核基质蛋白2抗体与恶性肿瘤相关性皮肌炎有关.此外抗转录中介因子1γ抗体和抗核基质蛋白2抗体还与皮肌炎其他一些特征性临床表型相关,如抗转录中介因子1γ抗体阳性的患者发生银屑病样损害、手掌角化过度性丘疹明显增加,而抗核基质蛋白2抗体阳性的患者发生前臂、小腿和颈部无力更为常见.抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体阳性患者发生间质性肺病风险和维持过长时间治疗的风险明显降低.因此临床上广泛地开展这些自身抗体的检测有助于指导皮肌炎的临床诊疗.     

免疫因素在特发性炎症性肌病中的作用机制

特发性炎症性肌炎包括多发性肌炎,皮肌炎及包涵体肌炎,是一组以肌肉组织的炎细胞浸润,肌无力为主要特点,浸润的炎细胞是以T淋巴细胞和巨噬细胞为主,个别也有B淋巴细胞。本文主要综述了T、B淋巴细胞、DC及其它APCs在IIMs中的病理生理作用。     

程序性细胞死亡蛋白1及其配体通路在特发性炎症性肌病中的研究进展

【摘要】 程序性细胞死亡蛋白（PD-1）与其配体（PD-L1）特异性结合后,具有共抑制/共刺激的免疫调控作用,可抑制T细胞活化、增殖及细胞因子分泌,参与肿瘤免疫、自身免疫及免疫耐受等。本文综述PD-1/PD-L1信号通路的组成结构、调控机制,以助于理解特发性炎症性肌病合并恶性肿瘤的免疫机制,寻找潜在的治疗靶点和诊断策略。     

Autoantibodies in sporadic porphyria cutanea tarda

In an investigation of autoimmune antibodies using indirect immunofluorescence and Western blot analysis in a group of porphyria cutanea tarda patients we did not find any cytosolic antibodies potentially able to inhibit uroporphyrinogen decarboxylase. Furthermore, no known etiological factors were present in any of our patients. We therefore consider the development of the recently reported autoantibody with a molecular weight of 40 kDa a reaction to infection with the hepatitis C virus. The origin of mostly antinuclear antibodies against liver antigens (50, 45 and 56 kDa), detected in seven patients, was not identified and their etiopathogenetic implications remain unknown.     

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium-201 scanning

Background It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis. Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes. Methods In this single blind study we used thallium-201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined. Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported. Conclusions Non-invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.     

慢性特发性荨麻疹免疫机制研究进展

慢性特发性荨麻疹的病因目前尚不明确。研究表明,慢性特发性荨麻疹发病大多有自身免疫机制参与,现已发现了数种与慢性特发性荨麻疹有关的自身抗体,其中抗FcεRI抗体、抗IgE抗体在慢性特发性荨麻疹发病中的地位尤为重要。B淋巴细胞刺激因子可能参与慢性特发性荨麻疹自身抗体的形成。另外,部分慢性特发性荨麻疹患者HLA-Ⅱ类抗原的等位基因DRB1 04频率增加,提示该病发病具有自身免疫遗传基础。     

慢性荨麻疹患者血清特异性IgE及甲状腺自身抗体的检测

目的：探讨自身免疫在特发性慢性荨麻疹和已知特异性过敏原慢性荨麻疹发病机制中的作用。方法：采用敏筛过敏原检测系统和放射免疫法，对120例慢性荨麻疹患进行血清特异性I姬及甲状腺自身抗体的检测。结果：86例血清特异性IgE阳性的慢性荨麻疹患中，23例(26．7％)甲状腺过氧化物酶抗体(TPOAb)阳性；18例(20．9％)促甲状腺受体自身抗体(TRAb)阳性。34例血清特异性IgE阴性的慢性荨麻疹患中，10例(29．4％)TPOAb阳性；8例(23．5％)TRAb阳性。两组慢性荨麻疹的TPOAb及TRAb均显高于正常对照组(P均＜0．01)；但两组之间的TPOAb及TRAb阳性率比较，均无显性差异(P均＞0．05)。结论：部分特发性慢性荨麻疹和已知特异性过敏原慢性荨麻疹的发病均可能有自身免疫机制参与。     

Autoantibodies to the heat-shock protein hsp73 in localized scleroderma

We determined the presence of antibodies to the heat-shock protein hsp73 (anti-hsp73) in 57 serum samples from patients with localized scleroderma using an enzyme-linked immunosorbent assay (ELISA). In addition, 30 samples from healthy individuals, 30 from patients systemic lupus erythematosus (SLE) and 32 from patients with systemic sclerosis were assessed. IgG and/or IgM anti-hsp73 antibodies were detected in 33% (19/57) of the patients with localized scleroderma. Among the three subtypes of localized scleroderma, generalized morphoea showed the highest incidence of anti-hsp73 antibodies (40%, 6/15). IgG and/or IgM anti-hsp73 antibodies were also detected in 9/30 samples (30%) from patients with SLE and in 13/32 samples (41%) from patients with systemic sclerosis, while the samples from the healthy controls were all negative for anti-hsp73. By immunoblotting, specific binding of antibodies to hsp73 was confirmed with representative serum samples that were positive for anti-hsp73 in the ELISA. Our findings indicate that the presence of anti-hsp73 is an additional immunological abnormality in localized scleroderma.     

皮肌炎伴发恶性肿瘤的研究进展

皮肌炎伴发恶性肿瘤是皮肌炎的一个亚型,伴发恶性肿瘤为皮肌炎预后不良的主要因素。恶性肿瘤高危发病时间为皮肌炎诊断前后1年内。与经典型皮肌炎相比,皮肌炎伴发恶性肿瘤具有患者年龄>50岁、Heliotrope征、皮肤坏死及肌炎特异性抗体抗转录中介因子1γ抗体、抗核基质蛋白2抗体阳性等典型的临床特点,这些特点提示需进行恶性肿瘤...