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Comparison of clinical features and outcomes of proliferative,fibrotic,and mixed subtypes of IgG4-related disease:A retrospective cohort study

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Chinese Medical Journal 2024年第3期137卷 303-311页

作者： Linyi Peng Xinlu Zhang Jiaxin Zhou Jieqiong Li Zheng Liu Hui Lu Yu Peng yunyun fei Yan Zhao Xiaofeng Zeng Wen Zhang Department of Rheumatology and Clinical Immunology Peking Union Medical College HospitalNational Clinical Research Center for Dermatologic and Immunologic DiseasesState Key Laboratory of Complex Severe and Rare DiseasesThe Ministry of Education Key LaboratoryBeijing 100730China Institute of Clinical Medicine Peking Union Medical College Hospital(PUMCH)Chinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100730China

Background:Immunoglobulin G4-related disease(IgG4-RD)is a recently recognized immune-mediated disorder that can affect almost any organ in the human ***4-RD can be categorized into proliferative and fibrotic subtypes based on patients’clinicopathological *** study aimed to compare the clinical manifestations,laboratory findings,and treatment outcomes of IgG4-RD among different ***:We prospectively enrolled 622 patients with newly diagnosed IgG4-RD at Peking Union Medical College Hospital from March 2011 to August *** patients were divided into three groups according to their clinicopathological characteristics:proliferative,fibrotic,and mixed *** compared demographic features,clinical manifestations,organ involvement,laboratory tests,and treatment agents across three *** then assessed the differences in treatment outcomes among 448 patients receiving glucocorticoids alone or in combination with ***,risk factors of relapse were revealed by applying the univariate and multivariate Cox regression ***:We classified the 622 patients into three groups consisting of 470 proliferative patients,55 fibrotic patients,and 97 mixed patients,*** found that gender distribution,age,disease duration,and frequency of allergy history were significantly different among *** terms of organ involvement,submandibular and lacrimal glands were frequently involved in the proliferative subtype,while retroperitoneum was the most commonly involved site in both fibrotic subtype and mixed *** comparison of laboratory tests revealed that eosinophils(P=0.010),total IgE(P=0.006),high-sensitivity C-reactive protein(P<0.001),erythrocyte sedimentation rate(P<0.001),complement C4(P<0.001),IgG(P=0.001),IgG1(P<0.001),IgG4(P<0.001),and IgA(P<0.001),at baseline were significantly different among three *** with proliferative and mixed subtypes,the fibrotic subtype showed the

关键词： IgG4-related disease Proliferative subtype Fibrotic subtype Clinical features Treatment outcome

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从经济结构转型角度看待我国健康体检行业发展前景

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中国卫生产业 2024年第2期21卷 249-252页

作者：马瑞苏绍荣费允云中国医学科学院北京协和医院北京100730

本文研究了健康体检行业在我国经济结构转型背景下的未来发展趋势和机遇。分析认为,健康体检行业是与人民健康和生活质量密切相关的产业,也是与经济发展和社会进步紧密相连的产业。作为服务业的重要组成部分,健康体检行业在我国经济结... 详细信息

本文研究了健康体检行业在我国经济结构转型背景下的未来发展趋势和机遇。分析认为,健康体检行业是与人民健康和生活质量密切相关的产业,也是与经济发展和社会进步紧密相连的产业。作为服务业的重要组成部分,健康体检行业在我国经济结构转型中将发挥重要作用。通过创新和改革,提高服务质量和效率,打造健康管理生态圈,利用新技术提升智能化和信息化水平,为健康体检行业的发展带来更多机遇,为我国经济结构转型和高质量发展做出贡献。

关键词：经济结构转型服务业增长健康体检行业发展前景

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稳定期IgG4相关性疾病患者减停药策略的研究

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罕见病研究 2024年第3期3卷 326-328页

作者：彭琳一聂玉雪费允云张文中国医学科学院北京协和医院风湿免疫科、国家皮肤免疫临床研究中心、疑难重症及罕见病全国重点实验室教育部重点实验室北京100730

2024年4月北京协和医院风湿免疫科张文教授团队在风湿病学领域顶级期刊Annals of the Rheumatic Diseases(中科院一区,影响因子为27.4)上以原创性论著形式发表了一项研究者发起的全国多中心随机对照研究探索稳定期IgG4相关性疾病(IgG4-r... 详细信息

2024年4月北京协和医院风湿免疫科张文教授团队在风湿病学领域顶级期刊Annals of the Rheumatic Diseases(中科院一区,影响因子为27.4)上以原创性论著形式发表了一项研究者发起的全国多中心随机对照研究探索稳定期IgG4相关性疾病(IgG4-related dis-ease,IgG4-RD)减停药策略^([1])。该研究提示,对于稳定期IgG4-RD患者,在预防复发方面,激素联合免疫抑制剂或单药免疫抑制剂的维持效果均显著优于停药组。该项研究填补了IgG4-RD维持治疗研究的空白,为该疾病的治疗领域提供了一项高质量循证医学证据。

关键词： IgG4相关性疾病临床研究免疫抑制剂糖皮质激素维持治疗

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糖代谢在类风湿关节炎发病中的作用

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中华临床免疫和变态反应杂志 2024年第2期18卷 164-168页

作者：马向波李镕利吴逊尧费允云邯郸市第一医院风湿免疫科河北邯郸056002 中国医学科学院北京协和医学院北京协和医院风湿免疫科北京100730

类风湿关节炎(rheumatoid arthritis,RA)是一种系统性炎症性自身免疫病,以慢性、对称性、破坏性多关节滑膜炎为特征。免疫系统的失调及炎症在其发病中发挥重要作用。近年来研究表明,糖代谢参与了免疫细胞的异常活化及炎症反应过程,与RA... 详细信息

类风湿关节炎(rheumatoid arthritis,RA)是一种系统性炎症性自身免疫病,以慢性、对称性、破坏性多关节滑膜炎为特征。免疫系统的失调及炎症在其发病中发挥重要作用。近年来研究表明,糖代谢参与了免疫细胞的异常活化及炎症反应过程,与RA的发病存在密切联系。本文将阐述RA糖代谢与T细胞、B细胞、巨噬细胞、炎症及胰岛素抵抗的关系,以及其在发病机制中的作用,可能为RA的治疗提供新思路。

关键词：类风湿关节炎糖代谢 T细胞 B细胞巨噬细胞炎症胰岛素抵抗

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The five major autoimmune diseases increase the risk of cancer:epidemiological data froma large-scale cohort study in China

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Cancer Communications 2022年第5期42卷 435-446页

作者： Ziyue Zhou Huazhen Liu Yiying Yang Jingya Zhou Lidan Zhao Hua Chen yunyun fei Wen Zhang Mengtao Li Yan Zhao Xiaofeng Zeng Fengchun Zhang Huaxia Yang Xuan Zhang Department of Rheumatology and Clinical Immunology National Clinical Research Center for Dermatologic and Immunologic Diseasesthe Ministry of Education Key LaboratoryPeking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730P.R.China Clinical Immunology Center Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730P.R.China Department of Medical Records Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730P.R.China State Key Laboratory of Difficult Severe and Rare DiseasesPeking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730P.R.China Department of Rheumatology National Center of GerontologyInstitute of Geriatric MedicineBeijing HospitalPeking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730P.R.China

Background:Cancer incidence and mortality have received critical attention during the long-term management of morbidities in patients with autoimmune diseases(AIDs).This study aimed to investigate and compare the risk of cancer associated with five major AIDs in a large-scale Chinese ***:A total of 8,120 AID patients consecutively admitted to a national tertiary referral center in China were included and followed-up for 38,726.55 patient-years,including those with systemic lupus erythematosus(SLE),rheumatoid arthritis(RA),Sjoren’s syndrome(SS),systemic scleroderma(SSc),and idiopathic inflammatory myositis(IIM).Demographic data,cancer incidence,predilecting sites and cancer onset time were recorded and compared among the five ***:Four hundred and thirty(5.3%)patients developed *** median agewas 57.5 years and AID durationwas 79.8 *** estimated total standardized incidence ratio(SIR)of cancer in AIDs patients was 3.37,with the highest SIR observed in IIM(4.31),followed by RA(3.99),SSc(3.77),SS(2.88)and SLE(2.58).The increased SIR of cancers in AID patients showed a female predominance(female ***:3.59 vs.2.77)and younger patient involvement(age<50 vs.≥50 years:4.88 vs.3.04).Patientswith SLE had increased SIRs for developing hematologic malignancies and solid tumors located in the urinary bladder,corpus uteri and cervix *** with SS had a significantly high SIR for developing non-Hodgkin’s *** 3 years of IIM diagnosis,74.6%of the patients developed cancer and they had a high risk of ovarian *** was associated with a wide distribution of scancers,including non-Hodgkin’s lymphoma,gynecologic,urinary tract,thyroid gland and lung *** patients had increased SIRs for developing cervical uterine,lung,and breast ***:Patients with five major AIDs in China had an increased risk of developing cancer,with a predominance in women and younger patients,although cancer incidence,predilection site

关键词： autoimmune disease cancer risk standardized incidence ratio lupus erythematosus rheumatoid arthritis Sjoren’s syndrome systemic scleroderma idiopathic inflammatory myositis epidemiology

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Proximity-enabled covalent binding of IL-2 to IL-2Rα selectively activates regulatory T cells and suppresses autoimmunity

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Signal Transduction and Targeted Therapy 2023年第2期8卷 795-812页

作者： Bo Zhang Jiaqi Sun Yeshuang Yuan Dezhong Ji Yeting Sun Yudong Liu Shengjie Li Xingxing Zhu Xunyao Wu Jin Hu Qiu Xie Ling Wu Lulu Liu Boyang Cheng Yuanjie Zhang Lingjuan Jiang Lidan Zhao fei Yu Wei Song Min Wang Yue Xu Shiliang Ma yunyun fei Lihe Zhang Demin Zhou Xuan Zhang State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College HospitalDepartment of RheumatologyBeijing HospitalNational Center of GerontologyInstitute of Geriatric MedicineChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China State Key Laboratory of Natural and Biomimetic Drugs School of Pharmaceutical SciencesPeking UniversityBeijing 100191China Department of Rheumatology Beijing HospitalNational Center of GerontologyInstitute of Geriatric MedicineClinical Immunology CenterGraduate School of Peking Union Medical CollegeChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Rheumatology and Clinical Immunology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeThe Ministry of Education Key LaboratoryBeijing 100730China

Interleukin-2(IL-2)is a pleiotropic cytokine that orchestrates bidirectional immune responses via regulatory T cells(Tregs)and effector cells,leading to paradoxical ***,we report a strategy that exploited genetic code expansion-guided incorporation of the latent bioreactive artificial amino acid fluorosulfate-L-tyrosine(FSY)into IL-2 for proximity-enabled covalent binding to IL-2Rαto selectively promote Treg *** found that FSY-bearing IL-2 variants,such as L72-FSY,covalently bound to IL-2Rαvia sulfur-fluoride exchange when in proximity,resulting in persistent recycling of IL-2 and selectively promoting the expansion of Tregs but not effector *** assessment of L72-FSY-expanded Tregs demonstrated that L72-FSY maintained Tregs in a central memory phenotype without driving terminal differentiation,as demonstrated by simultaneously attenuated expression of lymphocyte activation gene-3(LAG-3)and enhanced expression of programmed cell death protein-1(PD-1).Subcutaneous administration of L72-FSY in murine models of pristane-induced lupus and graft-versus-host disease(GvHD)resulted in enhanced and sustained therapeutic efficacy compared with wild-type IL-2 *** efficacy of L72-FSY was further improved by N-terminal PEGylation,which increased its circulatory retention for preferential and sustained *** proximity-enabled covalent binding strategy may accelerate the development of pleiotropic cytokines as a new class of immunomodulatory therapies.

关键词： sustained proximity consequences

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原发性胆汁性胆管炎与干燥综合征的关系

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临床肝胆病杂志 2021年第10期37卷 2262-2268页

作者：肖秋凤费允云中国医学科学院北京100730 北京协和医学院北京100730 北京协和医院风湿免疫科风湿免疫病学教育部重点实验室北京100730

原发性胆汁性胆管炎与干燥综合征都属于慢性自身免疫上皮细胞炎症,经常合并出现。旨在对原发性胆汁性胆管炎和干燥综合征的一般特点、临床表现、血清学特点、发病机制、治疗方案以及共同特征进行综述,探究二者共同的发病机制,为开发新... 详细信息

原发性胆汁性胆管炎与干燥综合征都属于慢性自身免疫上皮细胞炎症,经常合并出现。旨在对原发性胆汁性胆管炎和干燥综合征的一般特点、临床表现、血清学特点、发病机制、治疗方案以及共同特征进行综述,探究二者共同的发病机制,为开发新疗法提供思路,更好地管理患者。

关键词：肝硬化,胆汁性干燥综合征自身免疫疾病免疫稳态破坏

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Comparison of pathological findings between patients with relapsed and non-relapsed immunoglobin G4-related disease

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Chinese Medical Journal 2022年第4期135卷 465-467页

作者： Hui Lu Shan Wang Mu Wang Xiaowei Liu Linyi Peng Jiaxin Zhou Jieqiong Li Zheng Liu Xuan Luo Yu Peng yunyun fei Yan Zhao Xiaofeng Zeng Ruie Feng Wen Zhang Department of Rheumatology Peking Union Medical College HospitalChinese Academy of Medical Science and Peking Union Medical CollegeNational Clinical Research Center for Dermatologic and Immunologic DiseasesState Key Laboratory of Complex Severe and Rare DiseasesBeijing 100730China Department of Pathology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Stomatology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Ophthalmology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China

Immunoglobin(Ig)G4-related disease(IgG4-RD)is an immune-mediated fibroinflammatory condition characterized by elevated serum IgG4 levels and multi-organ *** characteristic histological findings of IgG4-RD are dense lymphoplasmacytic infiltration,fibrosis,and massive IgG4 positive plasma cells infiltrating the affected tissues.[1]Previous studies have demonstrated various relapse predictors corresponding to demographic,clinical characteristics,laboratory test results,and treatment ***,no study has revealed the risk factors of flares in *** study aimed to ascertain whether the pathological characteristics are related to therapeutic outcomes.

关键词： IgG4 relapse involvement

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系统性红斑狼疮伴周围神经病16例临床分析

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江苏医药 2016年第11期42卷 1235-1237页

作者：杨宁费允云南京中医药大学附属中西医结合医院江苏省中医药研究院风湿免疫科江苏省210028 中国医学科学院北京协和医院

目的总结系统性红斑狼疮(SLE)患者伴周围神经病的临床特征。方法回顾性分析16例SLE伴周围神经病的患者的临床资料。结果 16例患者中,对称性多神经病8例,多发性单神经病4例,单神经病1例,颅神经病变1例,自主神经病变2例。其中,四肢末端麻... 详细信息

目的总结系统性红斑狼疮(SLE)患者伴周围神经病的临床特征。方法回顾性分析16例SLE伴周围神经病的患者的临床资料。结果 16例患者中,对称性多神经病8例,多发性单神经病4例,单神经病1例,颅神经病变1例,自主神经病变2例。其中,四肢末端麻木9例,刺痛3例,烧灼样痛1例,痛觉过敏3例,肢体远端感觉减退9例,深感觉下降4例,肢体无力11例,足或腕下垂6例,肌肉萎缩3例,跟、膝腱反射减弱或消失5例。8例周围神经病患者同时合并有中枢神经系统受累。结论 SLE合并周围神经病感觉、运动常同时受累,可同时合并中枢神经系统病变。电生理检查可作为周围神经损害的早期诊断手段。

关键词：系统性红斑狼疮周围神经病

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组织蛋白酶S在原发性胆汁性胆管炎中作用的研究

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中华医学杂志 2019年第7期99卷 505-509页

作者：罗璇李霞杜志荣彭钰邓垂文费允云中国医学科学院北京协和医学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室北京100730 首都医科大学宣武医院风湿免疫科北京100053

目的探讨组织蛋白酶(Cat)S在原发性胆汁性胆管炎(PBC)中的作用。方法收集2016年8月至2017年7月于北京协和医院就诊的PBC及乙型肝炎(HBV)患者血清,2010年3月至2013年7月在北京协和医院住院的PBC患者肝脏穿刺标本。通过酶联免疫吸附法(... 详细信息

目的探讨组织蛋白酶(Cat)S在原发性胆汁性胆管炎(PBC)中的作用。方法收集2016年8月至2017年7月于北京协和医院就诊的PBC及乙型肝炎(HBV)患者血清,2010年3月至2013年7月在北京协和医院住院的PBC患者肝脏穿刺标本。通过酶联免疫吸附法(ELISA)检测PBC患者、HBV患者及健康对照血清总Cat S和pro-Cat S水平,计算活性形式active-Cat S,并分析PBC患者血清Cat S与胆汁淤积生化指标、IgM、IgG之的相关性;通过免疫荧光检测肝脏组织巨噬细胞浸润及Cat S表达。采用受试者工作特征曲线(ROC曲线)、t检验、Spearman相关性分析进行统计学分析。结果 PBC组患者血清总Cat S、pro-Cat S、active-Cat S表达水平均高于健康对照组和HBV组患者,差异有统计学意义(P<0.05)。PBC患者血清总-Cat S与碱性磷酸酶(ALP)、谷氨酰转肽酶(GGT)、IgG之间具有相关性(P<0.05);pro-Cat S与ALP、GGT相关(P<0.05)。采用受试者工作特征曲线分析发现,血清总Cat S、pro-Cat S和active-Cat S的曲线下面积(AUC)分别为0.85(P<0.01)、0.65(P<0.05)和0.77(P<0.01);最佳阈值分别为11.30、7.81及5.93 pg/L;敏感性和特异性分别为0.81和0.82、0.53和0.81、0.53和0.96。肝脏组织免疫荧光显示,PBC患者肝脏中存在巨噬细胞浸润,巨噬细胞和Cat S共表达占单个核细胞的平均百分比为23.77%。结论 PBC患者血清Cat S表达较健康对照和HBV患者明显增加,与血清IgG和ALP/GGT水平相关。肝脏组织巨噬细胞与Cat S共表达。Cat S可能参与PBC发病中抗原提呈过程。

关键词：原发性胆汁性胆管炎组织蛋白酶S 抗原提呈生物标记物

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