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...     

假性血管性水肿：一种新的皮肌炎皮疹

【摘要】 介绍一种新的皮肌炎皮疹类型——假性血管性水肿，其特点为患者面部、口唇、四肢为主出现局限性或弥漫性水肿，非凹陷性，可伴或不伴红斑，皮疹无明显瘙痒。大部分伴假性血管性水肿的皮肌炎患者抗转录中介因子1γ抗体阳性，会出现严重肌肉损伤，肌酶异常增高，以及难治性吞咽困难；少部分患者抗黑素瘤分化相关基因5抗体阳性，此类患者均有肺部累及。因此，皮肌炎患者出现假性血管性水肿时需要高度重视，抗体筛查有助于患者早期诊断和早期差异化治疗，改善患者的症状体征，提高患者的生存率。     

抗黑素瘤分化相关基因5抗体阳性皮肌炎合并快速进展性间质性肺病的临床特征及危险因素

目的：通过分析抗黑素瘤分化相关基因5抗体(MDA5)阳性皮肌炎(DM)患者合并快速进展性间质性肺病(RPILD和非RPILD的临床特点差异,探讨合并RPILD发生的危险因素。方法：回顾性分析南京医科大学炎性肌病及结缔组织病相关间质性肺病专病联盟组织收集的251例抗MDA5抗体阳性皮肌炎(MDA5+DM)患者临床资料,将其根据有无合并RPILD进行分组,采用t检验、U检验、χ²检验及Logistic回归分析合并RPILD患者的临床特点,探讨RPILD发生的危险因素。结果：将251例患者分为RPILD组(89例)和非RPILD组(162例),2组患者性别、年龄、病程、红细胞沉降率(ESR)、C反应蛋白(CRP)、抗Ro-52抗体阳性、铁蛋白(SF)、抗MDA5抗体滴度比较,差异均有统计学意义(P<0.05)。单因素Logistic回归分析显示年龄、ESR、抗MDA5抗体滴度、性别(男性)、CRP、抗Ro-52抗体均有统计学意义(OR>1,P<0.05);多因素Logistic回归分析显示性别(男性)、CRP、抗Ro-52抗体有统计学意义(OR>...     

抗核小体抗体在系统性红斑狼疮中的意义

狼疮中抗ds-DNA抗体、抗组蛋白抗体与核小体特异性抗体同属于大的抗核小体抗体家族,抗ds-DNA抗体、核小体特异性抗体和核小体-抗核小体抗体免疫复合物在系统性红斑狼疮的病理生理中起重要作用。抗核小体IgG抗体是比抗ds-DNA抗体更敏感的系统性红斑狼疮指标,抗核小体IgG抗体仅在系统性红斑狼疮、硬皮病、混合结缔组织病中出现,高水平的抗核小体IgG抗体几乎是仅在系统性红斑狼疮中出现,抗核小体IgG3抗体与系统性红斑狼疮疾病活动指数有很强的联系。     

伴手掌疼痛性皮疹的皮肌炎合并致死性急进性间质性肺病四例临床分析

目的 国内首报4例以手掌疼痛性皮疹为突出皮损表现的皮肌炎合并致死性急进性间质性肺病。方法 2014年12月至2017年4月,复旦大学附属中山医院皮肤科诊治4例具有手掌疼痛性皮疹的皮肌炎合并致死性急进性间质性肺病患者,分析其临床与病理特征。结果 患者中女3例,男1例,年龄47 ~ 59岁。4例中3例无肌肉受累。4例患者双手掌、指掌面和侧面均表现为多发红色坚实丘疹或结节,先于、晚于或与皮肌炎其他皮损同时发生。4例手掌疼痛性皮疹先于Ⅰ型呼吸衰竭3周至5个月发生。早期多误诊为接触性皮炎、湿疹或多形红斑。指掌侧皮损组织病理示真皮血管周围少量淋巴细胞浸润,血管、附属器周围数量不等的黏蛋白样物质沉积。4例中3例抗黑素瘤分化相关基因5抗体阳性。虽经抗炎和免疫抑制治疗,4例均死于呼吸衰竭。结论 皮肌炎患者伴发手掌疼痛性皮疹可能预示急进性间质性肺病发生,需及早对皮损活检以助识别,及时加强免疫抑制治疗,争取改善患者预后。     

皮肌炎/多发性肌炎患者并发间质性肺病的危险因素

目的 间质性肺病为皮肌炎和多发性肌炎的常见并发症之一，影响患者疗效和预后，探讨伴发间质性肺病的危险因素具有重要临床意义。方法 回顾性分析2019年6月—2021年11月于安徽医科大学第一附属医院住院治疗的132例皮肌炎和多发性肌炎患者，分为间质性肺病组和非间质性肺病组，并采用Logistic回归分析并发间质性肺病的独立危险因素。结果 132例患者中伴发间质性肺病的患者高达71例(53.79%)。单因素分析显示两组患者性别构成、年龄、眶周皮疹、Gottron丘疹、技工手发生率、抗PL-7、抗PMSCL-100、抗PMSCL-75、抗Mi2、抗SRP抗体阳性率、谷丙转氨酶、乳酸脱氢酶、CD4/CD8细胞计数、IL-6水平差异无统计学意义。发热、关节炎/痛、恶性肿瘤、抗核抗体、抗Jo-1抗体阳性率，血沉、C-反应蛋白、白细胞计数、中性粒细胞百分比、肌酸激酶、天冬氨酸氨基转移酶、癌胚抗原、糖类抗原153、铁蛋白水平在两组患者间差异有统计学意义(P<0.05)。Logistic回归分析提示抗Jo-1抗体和糖类抗原153为间质性肺病的独立危险因素。结论 间质性肺病为皮肌炎和多发性肌炎的常见并...     

脂溢性皮炎样皮肌炎：一种特殊类型的皮肌炎

介绍一种特殊类型的皮肌炎——脂溢性皮炎样皮肌炎,其特点为患者有面部脂溢性皮炎样皮疹,常伴有Gottron丘疹、反向Gottron丘疹、技工手和皮肤溃疡,很少累及肌肉。抗黑素瘤分化相关基因5抗体阳性的脂溢性皮炎样皮肌炎患者更易出现肺间质病变/急进性肺间质病变。通过皮疹联合抗体检测可对脂溢性皮炎样皮肌炎进行早期诊断,早期积...     

以间质性肺疾病为突出表现的皮肌炎

报告1例以间质性肺疾病（ILD）为突出表现的皮肌炎。患者女，66岁。咳嗽伴眼睑斑块、双手指斑疹半个月余。皮肤科检查：双侧眶周水肿性紫红色斑块，双手近端指间关节伸侧脱屑样斑疹，四肢肌力正常。实验室检查示抗黑色素瘤分化相关因子5(MDA5)抗体阳性，ILD进展迅速，患者呼吸困难。诊断：皮肌炎。治疗：予口服托法替布治疗后症状缓解。     

系统性红斑狼疮患者血清抗核小体抗体和脱氧核糖核酸酶Ⅰ活性的研究

目的：探讨血清抗核小体抗体和血清脱氧核糖核酸酶Ⅰ(deoxyribonuclease Ⅰ，DNase Ⅰ)的活性与系统性红斑狼疮(SLE)病情的关系。方法：采用间接ELISA法检测SLE患者血清抗核小体抗体浓度，DNA-甲基绿比色法测定其血清DNaseⅠ的活性。结果：SLE患者血清抗核小体抗体浓度明显高于正常对照，且与SLE病情活动程度、肾脏损害、血管炎及补体下降相关；SLE患者组血清DNase Ⅰ活性低于正常对照组，且与病情活动程度相关；血清抗核小体抗体浓度与DNase Ⅰ的活性成负相关。结论：抗核小体抗体是SLE的主要自身抗体之一，核小体刺激机体产生抗体的可能原因是DNase Ⅰ的活性下降。     

成人皮肌炎患者抗核抗体与临床特征及肿瘤风险的关系

目的 探讨成人皮肌炎患者抗核抗体与临床特征及肿瘤风险的关系.方法 回顾性分析2008年4月至2018年4月在苏州大学附属第一医院皮肤科住院的101例皮肌炎患者的临床资料,分为抗核抗体阳性组和阴性组,比较两组之间肌病、肿瘤发生风险以及其他临床特征的差异.92例患者随访2年.采用卡方检验分析比较两组的临床特征,利用多因素回...     

皮肌炎/临床无肌病性皮肌炎患者血清中CADM-140抗体的检测和临床意义

目的 对皮肌炎（DM）/临床无肌病性皮肌炎（CADM）患者进行CADM-140抗体检测,探讨CADM-140抗体与临床特征间的联系。方法 采集38例DM（22例）/CADM（16例）患者血清,另外采集46例伴有肺间质病变的其他结缔组织病患者血清,包括8例多发性肌炎、15例系统性红斑狼疮、5例系统性硬化病、6例干燥综合征、6例混合性结缔组织病、6例特发性肺纤维化和5例正常对照者。以重组黑素瘤分化相关基因5（rMDA-5）为底物,通过ELISA检测患者血清中CADM-140抗体,比较CADM-140抗体阳性与阴性患者的临床特征。 结果 ①16例CADM和22例DM患者血清CADM-140抗体阳性例数分别为7例和2例,CADM患者阳性率（43.8%）显著高于DM（9.1%）（P ＜ 0.05）,46例伴有肺间质病变的其他结缔组织病患者及5例正常人均阴性;②CADM-140抗体阳性患者皮肤溃疡和坏死的发生率为8/9,红细胞沉降率为（40.8 ± 23.1） mm/1 h,CADM-140抗体阴性组分别为6.9%和（22.5 ± 16.8） mm/1 h,两组比较,P ＜ 0.01和 ＜ 0.05;CADM-140抗体阳性患者乳酸脱氢酶水平显著高于阴性组（分别为328.3 ± 104.2和241.1 ± 100.3 IU/L,P ＜ 0.05）,而肌酸激酶显著低于阴性组（分别为156.3 ± 260.8和1806.2 ± 3737.1 IU/L,P ＜ 0.05）;两组间抗核抗体阳性率和恶性肿瘤发生率的差异无统计学意义;③CADM-140抗体阳性患者不仅肺间质病变发生率显著高于阴性组（分别为9/9和48.3%,P ＜ 0.01）,而且急进型肺间质病变发生率也显著高于阴性组（分别为5/9和0,P ＜ 0.05）。阳性组肺高分辨率CT评分（122.9 ± 54.8）显著高于阴性组（70.0 ± 59.8）（P ＜ 0.05）。结论 通过检测CADM-140抗体不仅可以判断DM/CADM是否合并肺间质病变,还可能是伴发急进型肺间质病变的血清学标记,动态观察血清CADM-140抗体水平也许有助于预测肺间质病变病程。     

Predictive factors for anti-MDA5 antibody in patients with dermatomyositis: a retrospective multicenter study

Background and objectives : Melanoma differentiation-associated gene 5 antibody (anti-MDA5) in dermatomyositis (DM) is associated with rapidly progressive interstitial lung disease and poor prognosis. Early diagnosis is key to improving the prognosis of these patients. The aim was to confirm cutaneous characteristics in patients with anti-MDA5 dermatomyositis and to explore new diagnostic markers for the presence of anti-MDA5 (anti-MDA5⁺). Patients and Methods : A multicenter cross-sectional retrospective cohort study of 124 patients diagnosed with DM, of which 37 were anti-MDA5⁺. Demographic data, laboratory data, and clinical manifestations were collected. Results : Anti-MDA5⁺ DM is characterized by a distinct mucocutaneous phenotype that includes oral lesions, alopecia, mechanic's hands, palmar and dorsal papules, palmar erythema, vasculopathy, and skin ulceration. We found vasculopathy and digit tip involvement very frequently in anti-MDA5⁺ patients (p <0.001), being a diagnostic marker of anti-MDA5⁺ (OR, 12.355; 95% CI 2.850–79.263; p  =  0.012 and OR, 7.447; 95% CI 2.103–46.718; p  =  0.004, respectively). The presence of ulcers deserves special mention, especially in anti-MDA5⁺ patients, because in our cohort, up to 97% of the anti-MDA5⁺ patients had ulcers. Conclusions : In patients with suspected DM with digit tip involvement or vasculopathy, the presence of anti-MDA5 antibodies must be ruled out, as it may be a clinical predictor.     

抗MDA5抗体阳性重症皮肌炎1例

患者女,31岁,多发肌肉疼痛4个月,咳嗽、气短2个月,发热3 d.肌电图提示肌炎活动期;特发性炎性肌炎谱示:抗MDA5抗体IgG(3+),抗Ro-52抗体IgG(3+);胸部CT提示两肺间质性改变及感染.诊断:皮肌炎,肺间质病变,I型呼吸衰竭,肺部感染.     

Clinical, serologic, and immunogenetic studies in patients with dermatomyositis.

Dermatomyositis is a disease of unknown etiology characterized by progressive, symmetrical, proximal muscle weakness with accompanying compatible cutaneous findings. Thirty-nine patients with dermatomyositis from the Louisville, Kentucky area were enrolled in this study. Patients were grouped into those with or without a malignancy. Ten patients (26%) either had or have had a malignancy. Twenty-five Caucasian patients were HLA typed for the A, B, DR and DQ locus antigens, of whom 5 had an associated malignancy and 20 did not have a malignancy. We found that no single antigen had a significantly increased or decreased frequency as compared with our control population for the entire group, or for any clinical subset we examined. Serologic testing revealed 4 patients with anti-Mi-2 antibodies and 1 patient with anti-PM-SCL antibodies. No patient had a positive anti-Jo-1 antibody in this group. The results of serologic tests in this group did not correlate with any clinical subset or HLA antigen. Our findings were in agreement with the previous reports in which approximately 25% of patients with DM have an associated malignancy. Our findings also support the notion that untargeted malignancy searches are not warranted. Contrary to previous reports we did not observe an inverse relationship between cancer and pulmonary disease in the dermatomyositis patient. This study does not indicate that there are any HLA associations or clinical associations, other than age, that distinguish patients with dermatomyositis as running a greater risk of developing malignancy.     

Analysis of dermatomyositis-specific autoantibodies and clinical characteristics in Japanese patients

Dermatomyositis (DM) is an idiopathic systemic inflammatory disease that is often accompanied by interstitial lung disease (ILD) or internal malignancy. New autoantibodies, anti-clinically amyopathic dermatomyositis 140 (anti-CADM-140) antibody (Ab) and anti-155/140 Ab, as well as anti-aminoacyl-tRNA synthetase (anti-ARS) Ab and anti-Mi-2 Ab, have been discovered and their utility indicated. However, the association between these autoantibodies and the clinical characteristics of DM is not fully understood, and it is unclear whether anti-155/140 Ab is "specific" to DM patients with internal malignancy. Therefore, we analyzed 55 DM patients and 18 non-DM patients with malignancy to evaluate the clinical characteristics, especially skin manifestations, in association with DM-specific autoantibodies detected by immunoprecipitation. Six patients (11%) had anti-CADM-140 Ab, nine (16%) had anti-155/140 Ab, eight (15%) had anti-ARS Ab and six (11%) had anti-Mi-2 Ab. The frequency of DM patients positive for any type of autoantibody was 53%. Among the 20 DM patients with ILD, three (15%) had both anti-CADM-140 Ab and rapidly progressive ILD, and required intensive therapy (P < 0.05). ILD found in anti-ARS Ab-positive patients did not progress rapidly. The prevalence of muscle involvement in patients with anti-CADM-140 Ab was 83%. Among the 18 DM patients with internal malignancy, four (22%) had anti-155/140 Ab, and internal malignancy was found in four cases (44%) of nine anti-155/140 Ab-positive patients. None of the non-DM patients with malignancy were positive for anti-155/140 Ab. In conclusion, the results of the present study indicate that anti-155/140 Ab is specific to DM patients with internal malignancy and that we may be able to predict prognosis of ILD and the presence of malignancy to some extent, suggesting that examination of autoantibodies in DM patients is clinically very useful. However, further investigation is needed because several findings differ from those of previous reports.     

Clinicopathological analysis of mechanic's hand associated with dermatomyositis

Mechanic's hand is often seen in the fingers of patients with dermatomyositis and is frequently associated with anti‐aminoacyl‐transfer RNA synthetase autoantibodies and interstitial lung disease. We analysed the clinical symptoms of 50 patients with dermatomyositis who had visited our department, 26 of whom also had mechanic's hand. A histological examination was carried out in 16 of the 26 cases, which revealed hyperkeratosis in all cases and colloid bodies in the epidermis in 15 cases. The number of cases of interstitial lung disease in patients with mechanic's hand (22/26, 85%) was significantly higher than that in those without mechanic's hand (12/24, 50%) (P < 0.05). Mechanic's hand is an important skin lesion of dermatomyositis, and increases the likelihood of interstitial lung disease     

The clinical spectrum of dermatomyositis and the new autoantibodies – with regard to two clinical cases with distinct associations

Dermatomyositis is a rare idiopathic inflammatory myopathy associated with different autoantibodies (anti-MDA5, anti-TIF1-γ) which are linked with typical and distinct phenotypes of dermatomyositis. We describe two cases that illustrate these diverse cutaneous and systemic manifestations.