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IgG4 Related Disease as a Differential Diagnosis in Multi-Organ Systemic Disease 认领
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作者 Mohammad Al Mousa Fahad Al Muhanna +1 位作者 Ahmad Al Shami Yaser M. Ali 《风湿病与自身免疫疾病期刊(英文)》 2021年第1期19-27,共9页
<div style="text-align:justify;"> <span style="font-family:Verdana;">IgG4 related disease is a relatively recently recognized systemic entity, that has been observed in nearly every org... <div style="text-align:justify;"> <span style="font-family:Verdana;">IgG4 related disease is a relatively recently recognized systemic entity, that has been observed in nearly every organ system, with similar histopathological features across all identified organs. IgG4 is an antibody molecule accounting for less than 5% of the total IgG count in a healthy person. The molecule is implicated in numerous autoimmune diseases;however its role in IgG4 related disease, whether primary or secondary, remains unknown. The gold standard in the diagnosis of this condition is demonstration of organ involvement from biopsy specimens. In this case report, the patient had exhibited symptoms related mainly to the gastrointestinal tract for 12 years prior to his diagnosis of IgG4 related disease. After this patient’s diagnosis of IgG4 related disease, he was commenced on Rituximab, a monoclonal antibody, which was successful in subsiding his symptoms and he has remained symptom free for the past two years of follow up since commencing treatment and hadn’t developed any complications of the disease nor any complications related to treatment. IgG4 related disease is a systemic autoimmune disease that involves multiple organs 60% - 90% of the time, therefore clinicians should be aware that IgG4 related disease can mimic some autoimmune rheumatic diseases and place it as a differential diagnosis when multi-organ disease with systemic features is involved.</span> </div> 展开更多
关键词 IgG4 Related Disease Autoimmune Disease RITUXIMAB
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Overlap of concurrent extrahepatic autoimmune diseases is associated with milder disease severity of newly diagnosed autoimmune hepatitis 认领
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作者 Tobias Mühling Helmut Rohrbach +1 位作者 Wolfgang Schepp Felix Gundling 《国际肝胆胰疾病杂志:英文版》 SCIE CAS 2021年第1期21-27,共7页
Background:Concurrent extrahepatic autoimmune disorders(CEHAID)are frequently observed in autoimmune hepatitis(AIH).It is not clear whether there is any prognostic significance of CEHAID on AIH.The aim of this study w... Background:Concurrent extrahepatic autoimmune disorders(CEHAID)are frequently observed in autoimmune hepatitis(AIH).It is not clear whether there is any prognostic significance of CEHAID on AIH.The aim of this study was to examine the prognostic impact of CEHAID and the correlation with the disease severity of AIH.Methods:This study included 65 hospitalized subjects who fulfilled the accepted criteria for AIH during an 8-year period(2009–2016).All records were manually screened for presence of associated autoimmune diseases.Disease severity of AIH was assessed by liver laboratory tests including the ratio of aspartate aminotransferase to alanine aminotransferase(AST/ALT)and liver histology.Results:Among the enrolled patients,52(80%)were female(median age 61 years,IQR 45–75).Fifty-six(86.2%)were classified as type-1 AIH.In 26(40%)patients at least one additional extrahepatic autoimmune disease was diagnosed.Thirty-four subjects were referred to our hospital because of acute presentation of AIH(supposed by an acute elevation of hepatic enzymes)for subsequent liver biopsy resulting in initial diagnosis of AIH.This group was stratified into 3 subgroups:(A)AIH alone(n=14);(B)overlap with primary biliary cirrhosis(PBC)/primary sclerosing cholangitis(PSC)(n=11);and(C)with CEHAID(n=9).AST/ALT ratio was the lowest in subgroup C(median 0.64,IQR 0.51–0.94;P=0.023),compared to subgroup A(median 0.91,IQR 0.66–1.10)and subgroup B(median 1.10,IQR 0.89–1.36).Patients with AIH alone showed a trend to the highest grade of fibrosis(mean 2.3;95%CI:1.5–3.0)with no statistical significance compared to subjects with CEHAID(lowest grade of fibrosis;mean 1.5;95%CI:0.2–2.8;P=0.380)whereas the ongoing inflammation was comparable.Conclusions:AST/ALT ratio and extent of fibrosis were lower in subjects with AIH and CEHAID,compared to subjects with only AIH.Therefore,the occurrence of CEHAID might be a predictor for lower disease severity of newly diagnosed acute onset AIH,possibly caused by an earlier diagnosis or different mode 展开更多
关键词 Autoimmune hepatitis Concurrent extrahepatic AUTOIMMUNE disease Serum aminotransferase activities
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Evaluation of controlled attenuation parameter in assessing hepatic steatosis in patients with autoimmune liver diseases 认领
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作者 Xi-Xi Ni Min Lian +9 位作者 Hui-Min Wu Xiao-Yun Li Li Sheng Han Bao Qi Miao Xiao Xiao Can-Jie Guo Hai Li Xiong Ma Jing Hua 《世界胃肠病学杂志:英文版》 SCIE CAS 2021年第1期80-91,共12页
BACKGROUND Hepatic steatosis commonly occurs in some chronic liver diseases and may affect disease progression.AIM To investigate the performance of controlled attenuation parameter(CAP)for the diagnosis of hepatic st... BACKGROUND Hepatic steatosis commonly occurs in some chronic liver diseases and may affect disease progression.AIM To investigate the performance of controlled attenuation parameter(CAP)for the diagnosis of hepatic steatosis in patients with autoimmune liver diseases(AILDs).METHODS Patients who were suspected of having AILDs and underwent liver biopsy were consistently enrolled.Liver stiffness measurement(LSM)and CAP were performed by transient elastography.The area under the receiver operating characteristic(AUROC)curve was used to evaluate the performance of CAP for diagnosing hepatic steatosis compared with biopsy.RESULTS Among 190 patients with biopsy-proven hepatic steatosis,69 were diagnosed with autoimmune hepatitis(AIH),18 with primary biliary cholangitis(PBC),and 27 with AIH-PBC overlap syndrome.The AUROCs of CAP for the diagnosis of steatosis in AILDS were 0.878(0.791-0.965)for S1,0.764(0.676-0.853)for S2,and 0.821(0.716-0.926)for S3.The CAP value was significantly related to hepatic steatosis grade(P<0.001).Among 69 patients with AIH,the median CAP score was 205.63±47.36 dB/m for S0,258.41±42.83 dB/m for S1,293.00±37.18 dB/m for S2,and 313.60±27.89 dB/m for S3.Compared with patients with nonalcoholic fatty liver disease(NAFLD)presenting with autoimmune markers,patients with AIH concomitant with NAFLD were much older and had higher serum IgG levels and LSM values.CONCLUSION CAP can be used as a noninvasive diagnostic method to evaluate hepatic steatosis in patients with AILDs.Determination of LSM combined with CAP may help to identify patients with AIH concomitant with NAFLD from those with NAFLD with autoimmune phenomena. 展开更多
关键词 Controlled attenuation parameter Hepatic steatosis Autoimmune liver diseases Nonalcoholic fatty liver disease Liver stiffness measurement Autoimmune hepatitis
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文章速递Progress in the Treatment of Hematological Diseases with Rituximab 认领
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作者 Xiangjun Fu Li’e Lin +6 位作者 Hongxia Yao Li Huang Can Meng Shuwen Wang Dan Liu Li Guo Mengling Duan 《血液病期刊(英文)》 2021年第1期6-14,共9页
Rituximab is a mouse and human chimeric CD<sub>20</sub> (anti-B cell) specific monoclonal antibody that has been approved by the U.S. Food and Drug Administration for the treatment of lymphoma. The express... Rituximab is a mouse and human chimeric CD<sub>20</sub> (anti-B cell) specific monoclonal antibody that has been approved by the U.S. Food and Drug Administration for the treatment of lymphoma. The expression of CD<sub>20</sub> antigen is expressed in the whole ontogeny of B cells, starting from the premature B cells in bone marrow to the differentiation of plasma cells in secondary lymphoid tissues. The wide distribution of CD<sub>20</sub> molecules allows rituximab to eliminate a large number of B cells. Rituximab is the core drug for the treatment of hematological diseases, often combined with drugs as a first-line treatment. Long-term hormone therapy often results in serious adverse reactions, and new therapies, which can avoid widespread immunotoxicity, have great potential for treating diseases of the blood system. 展开更多
关键词 Rituximab Malignant Lymphoma Leukemia Multiple Myeloma Autoimmune Diseases Hematopoietic Stem Cell Transplantation
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Negative conversion of autoantibody profile in chronic hepatitis B:A case report 认领
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作者 Xun Zhang Qin-Xiu Xie Dong-Mei Zhao 《世界临床病例杂志》 SCIE 2021年第5期1196-1203,共8页
BACKGROUND Autoimmune antibodies are detected in many diseases.Viral infections are accompanied by several immunopathological manifestations.Some autoimmune antibodies have been associated with the immune response ind... BACKGROUND Autoimmune antibodies are detected in many diseases.Viral infections are accompanied by several immunopathological manifestations.Some autoimmune antibodies have been associated with the immune response induced by virus or drugs.Thus,a comprehensive diagnosis of chronic hepatitis B combined with autoimmune hepatitis is required,and immunosuppressant or antiviral therapy should be carefully considered.CASE SUMMARY We present a case of a patient who had negative transformation of autoimmune antibodies during chronic active hepatitis B.A 50-year-old female who had a history of asymptomatic hepatitis B virus carriers for more than 10 years presented to the hospital with the complaint of weakness for 1 wk.Blood tests revealed elevated liver enzymes;the detection of autoantibodies was positive.Hepatitis B viral load was 72100000 IU/mL.The patient started tenofovir alafenamide fumigate 25 mg daily.Liver biopsy was performed,which was consistent with chronic active hepatitis B.The final diagnosis of the case was chronic active hepatitis B.The autoimmune antibodies turned negative after 4 wk of antiviral therapy.The patient recovered and was discharged with normal liver function.There was no appearance of autoantibodies,and liver function was normal at regular follow-ups.CONCLUSION Autoimmune antibodies may appear in patients with chronic active hepatitis.It is necessary to differentiate the diagnosis with autoimmune hepatitis. 展开更多
关键词 AUTOANTIBODIES Autoimmune hepatitis Chronic hepatitis B Immune responses Case report
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地塞米松联合甲巯咪唑治疗自身免疫性甲状腺炎效果及对T细胞免疫功能、炎性因子影响观察 认领
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作者 王翠英 任丽萍 +2 位作者 范琳 石巧 岳琳 《临床误诊误治》 2021年第1期31-36,共6页
目的观察地塞米松联合甲巯咪唑治疗自身免疫性甲状腺炎的效果及对T细胞免疫功能、炎性因子的影响。方法选取自身免疫性甲状腺炎79例,根据治疗方法不同将其分为观察组(41例)和对照组(38例)两组。观察组予地塞米松磷酸钠注射液联合甲巯咪... 目的观察地塞米松联合甲巯咪唑治疗自身免疫性甲状腺炎的效果及对T细胞免疫功能、炎性因子的影响。方法选取自身免疫性甲状腺炎79例,根据治疗方法不同将其分为观察组(41例)和对照组(38例)两组。观察组予地塞米松磷酸钠注射液联合甲巯咪唑片治疗,对照组予甲巯咪唑片治疗,疗程均为1个月。观察比较两组治疗后临床效果,治疗前后血清甲状腺功能相关指标、T细胞免疫功能相关指标和炎性因子水平,以及治疗期间不良反应发生情况。结果治疗后,观察组总有效率为90.24%高于对照组总有效率71.05%,差异有统计学意义(P<0.05)。治疗前,两组血清甲状腺功能相关指标、T细胞免疫功能相关指标和炎性因子水平比较差异均无统计学意义(P>0.05)。治疗后,血清游离三碘甲状腺原氨酸、甲状腺过氧化物酶抗体、甲状腺球蛋白抗体和肿瘤坏死因子-α、白细胞介素-17水平两组均较治疗前降低,且观察组低于对照组;两组血清CD4+水平及CD4+/CD8+值较治疗前升高,血清CD8+水平较治疗前降低,观察组血清CD4+水平及CD4+/CD8+值高于对照组,血清CD8+水平低于对照组,差异均有统计学意义(P<0.05或P<0.01)。治疗期间,观察组不良反应总发生率为34.15%高于对照组不良反应总发生率28.95%,但两组比较差异无统计学意义(P>0.05)。结论地塞米松联合甲巯咪唑治疗自身免疫性甲状腺炎可提高临床效果,降低自身抗体水平,改善机体免疫功能,减轻炎症反应程度,且安全性良好。 展开更多
关键词 甲状腺炎 自身免疫性 地塞米松 甲巯咪唑 游离三碘甲状腺原氨酸 促甲状腺激素 甲状腺过氧化物酶抗体 甲状腺球蛋白抗体 CD4+ CD8+ 肿瘤坏死因子-α 白细胞介素-17
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抗谷氨酸脱羧酶65抗体脑炎合并僵人综合征及自身免疫性多内分泌腺病综合征Ⅱ型一例并文献复习 认领
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作者 侯强强 章娟娟 +5 位作者 姬洋 郭媛媛 陈越 周农 汪凯 田仰华 《中华神经科杂志》 CAS 北大核心 2021年第2期112-116,共5页
目的报道1例抗谷氨酸脱羧酶65抗体脑炎合并僵人综合征(SPS)及自身免疫性多内分泌腺病综合征Ⅱ型(APS-Ⅱ)的病例,以提高对该疾病的认识。方法回顾性分析2017年至2019年安徽医科大学第一附属医院收治的1例34岁女性抗谷氨酸脱羧酶65抗体脑... 目的报道1例抗谷氨酸脱羧酶65抗体脑炎合并僵人综合征(SPS)及自身免疫性多内分泌腺病综合征Ⅱ型(APS-Ⅱ)的病例,以提高对该疾病的认识。方法回顾性分析2017年至2019年安徽医科大学第一附属医院收治的1例34岁女性抗谷氨酸脱羧酶65抗体脑炎合并SPS及APS-Ⅱ患者的临床资料并复习相关文献。结果患者2017年3月以近记忆缺损起病,脑脊液抗谷氨酸脱羧酶65抗体阳性,甲状腺抗体(抗甲状腺球蛋白抗体,甲状腺过氧化酶抗体)明显高于正常值;2018年10月出现肢体和躯干僵硬、活动受限;2019年7月发现血糖异常和甲状腺功能异常,糖尿病抗体(蛋白酪氨酸磷酸酶抗体,锌转运蛋白8抗体)阳性。诊断为抗谷氨酸脱羧酶65抗体脑炎、SPS、APS-Ⅱ(桥本甲状腺炎,1型糖尿病)。予以免疫治疗和对症治疗后,患者的症状稳定,未继续进展。结论抗谷氨酸脱羧酶65抗体脑炎合并SPS和APS-Ⅱ临床上较为罕见。抗谷氨酸脱羧酶65抗体或遗传易患性可能是该抗谷氨酸脱羧酶65抗体脑炎患者同时合并多种自身免疫疾病的原因。 展开更多
关键词 脑炎 自身免疫 僵人综合征 多内分泌腺疾病 自身免疫性 抗谷氨酸脱羧酶65抗体
Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis 认领
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作者 Francisco J.Barrantes 《中国神经再生研究:英文版》 SCIE CAS 2021年第2期242-246,共5页
Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the m... Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine receptor.Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse.This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis.The review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional dynamics.The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis. 展开更多
关键词 AGRIN autoimmune diseases muscle end-plate muscle specific kinase MuSK myasthenia gravis NANOSCOPY neuromuscular junction nicotinic acetylcholine receptor RAPSYN superresolution microscopy
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小分子脾酪氨酸激酶抑制剂临床研究进展 认领
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作者 范露 崔兵兵 +1 位作者 陆涛 陈亚东 《药学进展》 CAS 2021年第1期44-54,共11页
脾酪氨酸激酶(Syk)是一种细胞质非受体蛋白酪氨酸激酶,主要在造血细胞中表达。Syk不仅在免疫受体信号中扮演至关重要的角色,同时还调节其他不同的生物功能,包括细胞的黏附、增殖、破骨细胞成熟、血小板活化等方面。近年来Syk在血液肿瘤... 脾酪氨酸激酶(Syk)是一种细胞质非受体蛋白酪氨酸激酶,主要在造血细胞中表达。Syk不仅在免疫受体信号中扮演至关重要的角色,同时还调节其他不同的生物功能,包括细胞的黏附、增殖、破骨细胞成熟、血小板活化等方面。近年来Syk在血液肿瘤、自身免疫、炎症疾病等方向已经成为极具吸引力的药物靶标。通过介绍Syk的结构与激活机制,及其信号通路与疾病间关系,对口服小分子Syk抑制剂的临床研究进展进行综述,为其进一步研究与应用提供参考。 展开更多
关键词 脾酪氨酸激酶 抑制剂 自身免疫 血液肿瘤
硒酵母联合维生素D对实验性自身免疫甲状腺炎大鼠甲状腺相关激素及抗体的影响 认领
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作者 侯丽萍 耿建林 +1 位作者 谷巍 刘晴晴 《天津医药》 CAS 北大核心 2021年第2期136-141,共6页
目的观察硒酵母联合维生素D对实验性自身免疫性甲状腺炎(EAT)大鼠甲状腺相关激素及抗体的作用。方法55只雌性SD大鼠,其中45只采用猪甲状腺球蛋白(pTG)致敏+饮用高碘水建立EAT大鼠模型,建模成功大鼠(40只)采用随机数字表法分为模型组、... 目的观察硒酵母联合维生素D对实验性自身免疫性甲状腺炎(EAT)大鼠甲状腺相关激素及抗体的作用。方法55只雌性SD大鼠,其中45只采用猪甲状腺球蛋白(pTG)致敏+饮用高碘水建立EAT大鼠模型,建模成功大鼠(40只)采用随机数字表法分为模型组、硒酵母组、维生素D组、联合组,每组10只;其余10只为对照组。加强免疫4周后,硒酵母组予硒酵母溶液灌胃+腹腔注射生理盐水,维生素D组予生理盐水灌胃+腹腔注射维生素D3注射液,联合组予硒酵母溶液灌胃+腹腔注射维生素D3注射液,对照组和模型组予生理盐水灌胃+腹腔注射生理盐水。6周后,比较各组血清游离三碘甲状腺原氨酸(FT3)、游离甲状腺素(FT4)、促甲状腺激素(TSH)、甲状腺球蛋白抗体(TGAb)、甲状腺过氧化物酶抗体(TPOAb)、干扰素-γ(IFN-γ)、白介素(IL)-4、IFN-γ/IL-4;HE染色比较各组甲状腺组织病理学形态;比较各组甲状腺组织p38MAPK、环氧合酶-2(COX-2)mRNA相对表达量及p38MAPK、pp38MAPK、COX-2蛋白相对表达量。结果HE染色结果显示,对照组甲状腺滤泡形态正常;模型组甲状腺滤泡结构破坏,淋巴细胞浸润,间质纤维化;硒酵母组、维生素D组及联合组较模型组均有一定改善,其中联合组改善最为明显。与模型组比较,硒酵母组、维生素D组、联合组血清FT3、FT4、TSH、TGAb、TPOAb、IFN-γ、IFN-γ/IL-4水平,甲状腺组织COX-2 mRNA相对表达量及p-p38MAPK、COX-2蛋白相对表达量均降低(P<0.05),血清IL-4水平均升高(P<0.05);与硒酵母组和维生素D组比较,联合组血清FT3、FT4、TSH、TGAb、TPOAb、IFN-γ、IFN-γ/IL-4水平降低(P<0.05),甲状腺组织COX-2 mRNA相对表达量及p-p38MAPK、COX-2蛋白相对表达量均降低,血清IL-4水平升高(P<0.05)。结论硒酵母联合维生素D可有效抑制EAT大鼠甲状腺组织损伤,保护甲状腺组织,其机制可能是通过抑制MAPK信号通路发挥调控作用。 展开更多
关键词 甲状腺炎 自身免疫性 维生素D 大鼠 Sprague-Dawley 甲状腺激素类 Th1-Th2平衡 P38丝裂原活化蛋白激酶类 硒酵母
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熊去氧胆酸联合糖皮质激素治疗伴自身免疫性肝炎特征原发性胆汁性胆管炎的效果分析 认领
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作者 杨宁 田思远 +3 位作者 张苗 马硕怡 刘雁声 韩英 《解放军医药杂志》 CAS 2021年第3期72-77,共6页
目的对比观察伴自身免疫性肝炎(autoimmune hepatitis,AIH)特征原发性胆汁性胆管炎(primary biliary cholangitis,PBC)患者采用熊去氧胆酸(UDCA)单药治疗和UDCA联合糖皮质激素治疗的效果差异。方法选取我院2008年12月—2019年12月收治的... 目的对比观察伴自身免疫性肝炎(autoimmune hepatitis,AIH)特征原发性胆汁性胆管炎(primary biliary cholangitis,PBC)患者采用熊去氧胆酸(UDCA)单药治疗和UDCA联合糖皮质激素治疗的效果差异。方法选取我院2008年12月—2019年12月收治的178例伴AIH特征PBC患者,根据治疗方式的不同分为UDCA联合糖皮质激素组(联合治疗组)33例和UDCA单药治疗组(单药治疗组)145例,比较不同治疗方案的疗效差异,并用Logistic回归分析探讨与治疗应答相关的影响因素。结果2组治疗1年后丙氨酸转氨酶(ALT)、天冬氨酸转氨酶、碱性磷酸酶(ALP)、谷氨酰转移酶(GGT)、免疫球蛋白M均显著下降(P<0.05或P<0.01),血清球蛋白(GLB)、免疫球蛋白G(IgG)治疗前后比较差异无统计学意义(P>0.05),但联合治疗组治疗1年后GLB、IgG下降,单药治疗组上升。2组仅GGT、IgG、GLB在部分月份比较差异有统计学意义(P<0.05或P<0.01)。联合治疗组ALP、ALT、IgG生化应答率及组织学缓解率虽均高于单药治疗组,但差异无统计学意义(P>0.05)。多因素Logistic回归分析提示ALP、IgG、抗Ro-52抗体是伴AIH特征PBC患者完全生化应答的影响因素(P<0.05或P<0.01),抗SSA抗体是其组织学缓解的影响因素(P<0.01)。结论UDCA联合糖皮质激素治疗伴AIH特征PBC的效果与UDCA单药治疗无显著差异;对于UDCA单药治疗应答不佳的伴AIH特征PBC患者可考虑联合糖皮质激素治疗。 展开更多
关键词 肝炎 自身免疫性 胆管炎 胆汁性 合并症 熊去氧胆酸 糖皮质激素 治疗结果 影响因素分析
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伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征 认领
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作者 杨宁 孙可帅 +3 位作者 田思远 刘雁声 贾桂 韩英 《解放军医学杂志》 CAS 北大核心 2021年第1期36-41,共6页
目的分析伴自身免疫性肝炎(AIH)特征的原发性胆汁性胆管炎(PBC)患者的临床特征。方法纳入2008年12月-2018年12月空军军医大学附属西京医院消化内科确诊的PBC患者461例,根据PBC-AIH重叠综合征(PBC-AIH OS)、AIH-PBC及PBC的诊断标准分为PB... 目的分析伴自身免疫性肝炎(AIH)特征的原发性胆汁性胆管炎(PBC)患者的临床特征。方法纳入2008年12月-2018年12月空军军医大学附属西京医院消化内科确诊的PBC患者461例,根据PBC-AIH重叠综合征(PBC-AIH OS)、AIH-PBC及PBC的诊断标准分为PBC-AIH OS组(n=50)、AIH-PBC组(n=178)与单纯PBC组(n=233),分析各组一般临床症状(黄疸、乏力、瘙痒等)、生化免疫指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、免疫球蛋白G/M(IgG、IgM)、总/直接胆红素(TBIL、DBIL)、白/球蛋白(ALB、GLB)]、自身抗体[抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(ASMA)]、肝脏炎症纤维化分级分期、基于4因子的纤维化指数(FIB-4)和天门冬氨酸氨基转移酶/血小板比值指数(APRI),以及5年不良事件累积发生率等。结果 AIH-PBC组及单纯PBC组黄疸、纳差发生率均明显低于PBC-AIH OS组(P<0.05)。各组ALT、AST、TBIL、IgG、IgM、GLB水平差异有统计学意义(P<0.05),多以AIH-PBC组及PBC-AIH OS组较高。自身抗体检测结果显示,AIH-PBC组SSA、Ro-52抗体检出率明显高于单纯PBC组,ASMA、可溶性肝抗原/肝胰抗原抗体(SLA/LP)、SSB检出率明显低于PBC-AIH OS组(P<0.05)。AIH-PBC组IgG、IgM水平高于单纯PBC组(P<0.05)。AIH-PBC组和PBC-AIH OS组肝组织病理分级分期及FIB-4、APRI指数明显高于单纯PBC组(P<0.05)。5年累积不良事件发生率PBC-AIH OS组明显高于AIH-PBC组,AIH-PBC组明显高于单纯PBC组(P<0.05)。结论 AIH-PBC患者在临床表现上与PBC-AIH OS及单纯PBC患者有较大差异,长期预后较单纯PBC患者差。 展开更多
关键词 原发性胆汁性胆管炎 肝炎 自身免疫性 临床特征
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Targeting metabolism to treat multiple sclerosis 认领
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作者 Pavan Bhargava 《中国神经再生研究:英文版》 SCIE CAS 2021年第3期502-503,共2页
Multiple sclerosis(MS)is a chronic autoimmune disorder of the central nervous system(CNS)that has both inflammatory and neurodegenerative com ponents.Both genetic and environmental factors confer risk for developing t... Multiple sclerosis(MS)is a chronic autoimmune disorder of the central nervous system(CNS)that has both inflammatory and neurodegenerative com ponents.Both genetic and environmental factors confer risk for developing the disease.Additionally,gut microbiota appears to play an important role in the risk for MS and potentially also in affecting disease severity,though our understanding of these interactions are still incomplete(Bhargava and Mowry,2014). 展开更多
关键词 METABOLISM SCLEROSIS AUTOIMMUNE
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自身免疫性胰腺炎临床特点分析 认领
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作者 雷鹏 董小英 +2 位作者 卜稳平 王根旺 于松宁 《宁夏医科大学学报》 2021年第2期190-194,共5页
目的总结自身免疫性胰腺炎(autoimmune pancreatitis,AIP)的临床特点与诊治方法。方法收集27例AIP患者的临床资料,对其临床症状、影像学特点、血清学检查及组织病理学结果进行分析总结。结果27例患者中,22例(81.5%)以梗阻性黄疸为主要... 目的总结自身免疫性胰腺炎(autoimmune pancreatitis,AIP)的临床特点与诊治方法。方法收集27例AIP患者的临床资料,对其临床症状、影像学特点、血清学检查及组织病理学结果进行分析总结。结果27例患者中,22例(81.5%)以梗阻性黄疸为主要临床表现;19例(70.4%)CT检查显示全胰弥漫性肿大,胰腺有明显低密度肿块者8例(29.6%);23例检测IgG4,阳性者19例(82.6%);根据临床症状、影像学表现、血清学及组织病理学确诊17例(63.0%),糖皮质激素诊断性治疗确诊4例(14.9%),6例(22.2%)经手术探查确诊;6例(22.2%)患者术前误诊为胰腺癌或胆管癌,行手术治疗;诊断明确的患者除2例外其余均行受正规的糖皮质激素治疗(口服泼尼松),随访期4~60个月,2例复发,继续激素治疗半年后缓解。结论AIP少见且缺乏特异性的临床表现,误诊率较高,足够重视、充分认识及综合分析临床资料是诊断的关键。 展开更多
关键词 胰腺炎 自身免疫性 诊断 激素治疗
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microRNA在自身免疫性肝病中的作用 认领
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作者 蔡萌强 刘素彤 +2 位作者 刘君颖 张丽慧 赵文霞 《临床肝胆病杂志》 CAS 北大核心 2021年第1期212-215,共4页
microRNA(miRNA)通过抑制靶基因转录后翻译,影响细胞分化、增殖与凋亡等生物学过程,广泛参与调节生物体内的免疫与炎症反应。自身免疫性肝病是一种异常免疫介导的肝胆系统慢性炎症性疾病,miRNA参与的肝组织异常免疫炎症反应与自身免疫... microRNA(miRNA)通过抑制靶基因转录后翻译,影响细胞分化、增殖与凋亡等生物学过程,广泛参与调节生物体内的免疫与炎症反应。自身免疫性肝病是一种异常免疫介导的肝胆系统慢性炎症性疾病,miRNA参与的肝组织异常免疫炎症反应与自身免疫性肝病的发生发展过程密切相关。现就当前miRNA在自身免疫性肝病中的研究进展作一综述。 展开更多
关键词 微RNAS 肝炎 自身免疫性 肝硬化 胆汁性 胆管炎 硬化性
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Bamboo Nodes of Vocal Fold — A Clinical Study of 14 Cases 认领
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作者 Yi Wang Chanyuan Wu Qian Wang 《中国医学科学杂志:英文版》 CAS 2021年第1期43-49,共7页
Objective Bamboo node(BN)of vocal folds is a rare disease which is mostly associated with autoimmune diseases and always involves women as reported in literature.The purpose of this study is to investigate the clinica... Objective Bamboo node(BN)of vocal folds is a rare disease which is mostly associated with autoimmune diseases and always involves women as reported in literature.The purpose of this study is to investigate the clinical characteristics and the treatments in a series of BN cases.Methods We retrospectively collected and reviewed clinical information of all patients who presented bamboo nodes by fibreoptic laryngoscopy from 2011 to 2020 in our hospital,including the demographic information,clinical symptoms,associated autoimmune diseases,voice abuse,treatments,and outcomes.Patients were followed up periodically at clinics with fibreoptic laryngoscopy and/or voice recovery evaluation using the selt assessed voice visual analog scale(VAS).Results Tdtally 14 patients were diagnosed with BN and were included in the study.BN involved both women(8/14,57.1%)and men(6/14,42.9%).Bilateral lesions were found in 13 patients(92.9%).The most common symptom of BN was hoarseness(100%).Ten(71.4%)of 14 patients experienced voice abuse.Thirteen(92.9%)of them were concomitant with autoimmunity disease,and received medication therapy for the underlying autoimmune diseases;one patient received anti-acid treatment for the gastroesophageal reflux disease.Voice rests were performed in all patients.Voice recovered completely in 3 cases(21.4%),improved remarkably in 8 cases(57.1%),improved slightly in 1 case(7.1%),and no improvement in 2 cases(14.3%).Conclusions BNs are mostly associated with autoimmune disease and vocal abuse.It may occur in both females and males・Conservative treatment for the underlying autoimmune diseases and vocal rest are beneficial to voice recovery in most of the patients,and can be used as the first choice of treatment. 展开更多
关键词 bamboo nodes autoimmune disease HOARSENESS voice abuse conservative treatment
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Nephrotic syndrome in syngeneic hematopoietic stem cell transplantation recipients: A case report 认领
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作者 Ming-Chuan Bai Jing-Jing Wu +2 位作者 Kou-Rong Miao Jing-Feng Zhu Hui-Juan Mao 《世界临床病例杂志》 SCIE 2021年第3期614-622,共9页
BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still... BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still seriously affect the quality of life and even threaten the lives of patients.There is evidence that glomerular diseases can manifest as GVHD.However,GVHD should not occur as a result of syngeneic HSCT.CASE SUMMARY A 20-year-old male diagnosed with T lymphoblastic lymphoma(stage IIIA,aaIPI 1)in September 2013 was treated with six cycles of hyper-CVAD and achieved complete remission.He underwent syngeneic HSCT in June 2014,and had no kidney disease history before the transplant.However,nephrotic syndrome occurred 24 mo later in the patient after syngeneic HSCT.Renal biopsy was performed,which led to a diagnosis of atypical membranous nephropathy.After treatment with glucocorticoids combined with cyclophosphamide and cyclosporine,the nephrotic syndrome was completely relieved.CONCLUSION We report a case of delayed nephrotic syndrome after syngeneic HSCT.Antibodymediated autoimmune glomerular disease may be the underlying mechanism.After treatment with immunosuppressive agents,the nephrotic syndrome was completely relieved but further long-term follow-up is still needed. 展开更多
关键词 Syngeneic hematopoietic stem cell transplantation Nephrotic syndrome Graft vs host disease CYCLOSPORINE Autoimmune glomerular disease Case report
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A long and winding road to understand latent autoimmune diabetes in adults 认领
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作者 Shuo-Ming Luo Bing-Wen Liu +2 位作者 Wen-Feng Yin Xia Li Zhi-Guang Zhou 《中华医学杂志:英文版》 SCIE CAS 2021年第1期1-4,共4页
Latent autoimmune diabetes in adults(LADA)describes a subgroup of patients who develop phenotypic type 2 diabetes(T2D)but with markers of islet autoimmunity.It is under debate whether LADA is a subtype of type 1 diabe... Latent autoimmune diabetes in adults(LADA)describes a subgroup of patients who develop phenotypic type 2 diabetes(T2D)but with markers of islet autoimmunity.It is under debate whether LADA is a subtype of type 1 diabetes(T1D)or a unique disease.LADA comprises about 1.5%to 14.2%in phenotypic T2D depending on ethnicity.It is the most common form of autoimmune diabetes diagnosed in adults,with 3.3-to 12.2-fold higher prevalence than that of adult-onset T1D. 展开更多
关键词 AUTOIMMUNE DIABETES ISLET
文章速递Application of single-cell sequencing in autoimmune diseases 认领
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作者 Liang-Yu Mi Jin-Fang Gao +3 位作者 Dan Ma Li-Yun Zhang Gai-Lian Zhang Ke Xu 《中华医学杂志:英文版》 SCIE CAS CSCD 2021年第4期495-497,共3页
To the Editor:The Human Genome Project decodes the mysteries of life,explores the pathogenesis of disease,and provides scientific evidence for the diagnosis and treatment of disease.Traditional sequencing methods pred... To the Editor:The Human Genome Project decodes the mysteries of life,explores the pathogenesis of disease,and provides scientific evidence for the diagnosis and treatment of disease.Traditional sequencing methods predominantly provide genome-wide sequence information and an average of the mix of cell types,which does not take into account the heterogeneity between cells and between subtypes.Advances in genome sequencing technology have allowed the development of single cell sequencing(SCS). 展开更多
关键词 diagnosis diseases autoimmune
文章速递受体相互作用蛋白3介导自身免疫性肝炎肝脏单核细胞来源巨噬细胞募集 认领
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作者 刘曼 张红霞 +1 位作者 周璐 王邦茂 《中华消化杂志》 CAS CSCD 北大核心 2021年第1期35-42,共8页
目的探索受体相互作用蛋白3(RIP3)对自身免疫性肝炎(AIH)肝脏单核细胞来源巨噬细胞浸润的调控作用。方法纳入2018年1至6月于天津医科大学总医院消化科行肝穿刺活组织病理学检查的AIH患者10例,同期选择年龄和性别均匹配且无肝功能异常的... 目的探索受体相互作用蛋白3(RIP3)对自身免疫性肝炎(AIH)肝脏单核细胞来源巨噬细胞浸润的调控作用。方法纳入2018年1至6月于天津医科大学总医院消化科行肝穿刺活组织病理学检查的AIH患者10例,同期选择年龄和性别均匹配且无肝功能异常的5例肝囊肿患者作为对照,应用免疫荧光染色观察AIH患者和对照者肝组织单核细胞来源巨噬细胞浸润情况。将Raw264.7巨噬细胞分为对照组、脂多糖组、脂多糖+RIP3抑制剂GSK872(GSK872)组,采用定量聚合酶链反应(qPCR)检测巨噬细胞RIP3、混合谱系蛋白激酶样结构域(MLKL)、TNF-α、IL-6、IL-1β、细胞炎性小体Nod样蛋白3(NLRP3)、CC趋化因子配体(CCL)2和CCL5的mRNA水平;将Raw264.7巨噬细胞分为对照组、脂多糖组、脂多糖+地塞米松组,采用qPCR检测巨噬细胞TNF-α、NLRP3、RIP3和MLKL的mRNA水平。选择24只6周龄雌性C57BL/6小鼠建立急性AIH小鼠模型,并将其分为对照组、刀豆蛋白A(ConA)组、ConA+地塞米松组和ConA+GSK872组(每组6只),处死小鼠后收集外周血和肝组织,观察小鼠肝脏病理学表现,测定血清ALT和AST水平,采用qPCR检测CCL2和CC趋化因子受体2(CCR2)的mRNA水平,采用流式细胞术分析小鼠肝脏巨噬细胞比例。统计学方法采用独立样本t检验和单因素方差分析。结果AIH患者肝脏CD68阳性组织驻留巨噬细胞(库普弗细胞)和MAC387阳性单核细胞来源巨噬细胞比例均高于对照者[(0.84±0.21)%比(0.09±0.03)%、(0.79±0.13)%比(0.03±0.01)%],差异均有统计学意义(t=3.00、4.84,P均<0.05)。脂多糖组巨噬细胞内RIP3、MLKL、TNF-α、IL-6、IL-1β、NLRP3、CCL2、CCL5的mRNA水平均高于对照组和脂多糖+GSK872组(1.64±0.16比1.07±0.07和0.63±0.11,10.45±1.37比1.10±0.33和1.51±0.63,5.43±0.59比0.94±0.06和2.59±0.45,204.20±30.73比1.26±0.19和111.40±11.62,20848.00±362.00比1.09±0.26和10940.00±566.60,7.47±1.17比1.09±0.09和3.79±0.89,68.03±5.15比1.14±0.19和14.09±2.62,5935.12±96.20比1.43±0.46和673.50±49.10),差异均有统计学意义(t=3.11、5.21,6.65、6.55,7.57、3.96,6.60、3.06,8.83、4.08,5.46、2.56,12.97、10.16,25.34、14.99;P均<0.05)。脂多糖组巨噬细胞TNF-α、NLRP3、RIP3和MLKL的mRNA水平均高于对照组和脂多糖+地塞米松组(8.85±1.43比1.44±0.43和3.63±0.63,6.42±0.86比0.99±0.12和2.07±0.17,1.72±0.21比0.93±0.09和0.43±0.07,6.87±0.85比1.62±0.31和1.41±0.29),差异均有统计学意义(t=4.95、3.33,6.24、4.95,3.04、5.11,5.77、6.07;P均<0.05)。ConA组小鼠肝脏表现出明显的炎症细胞浸润和点状坏死。ConA组小鼠的血清ALT和AST水平均高于对照组、ConA+地塞米松组和ConA+GSK872组[(2569.00±45.44)U/L比(49.38±9.07)、(103.00±14.07)和(759.30±34.99)U/L,(3335.00±88.79)U/L比(108.50±18.10)、(460.00±97.40)和(1573.85±36.06)U/L],且ConA+地塞米松组小鼠的血清ALT和AST水平均低于ConA+GSK872组,差异均有统计学意义(t=5.54、5.42、3.90、4.63、4.16、3.79、6.70、2.71,P均<0.05)。ConA组小鼠肝脏CCL2和CCR2的mRNA水平均高于对照组、ConA+地塞米松组和ConA+GSK872组(92.64±10.57比0.78±0.15、5.64±1.00和9.47±2.06,5.73±0.39比0.98±0.22、2.18±0.22和2.98±0.33),差异均有统计学意义(t=7.66、7.24、5.87、8.71、8.58、5.45,P均<0.01)。ConA组小鼠肝脏CD45^(+)CD11b^(+)F4/80^(+)总巨噬细胞比例和CD45^(+)CD11b^(hi)F4/80^(lo)浸润的单核巨噬细胞比例均高于对照组、ConA+地塞米松组和ConA+GSK872组(0.86±0.02比0.73±0.03、0.68±0.02和0.72±0.03,0.56±0.02比0.08±0.02、0.11±0.01和0.08±0.01),CD45^(+)CD11b^(lo)F4/80^(hi)肝脏驻留巨噬细胞(库普弗细胞)比例低于对照组、ConA+地塞米松组和GSK872组(0.24±0.03比0.58±0.04、0.52±0.07和0.56±0.07),差异均有统计学意义(t=4.27、5.90、3.89,18.70、19.87、20.52,7.35、3.82、3.87,P均<0.05)。结论AIH患者肝脏巨噬细胞数量增加。RIP3信号介导免疫性肝炎肝脏单核细胞来源巨噬细胞浸润并可能成为AIH的潜在治疗靶点。 展开更多
关键词 肝炎 自身免疫性 巨噬细胞 受体相互作用蛋白3 细胞因子类 趋化因子
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