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Hemophagocytic lymphohistiocytosis triggered by relapsing polychondritis:A case report

World Journal of Orthopedics 2024年第8期15卷 813-819页

作者： Mi-Ran Han Jeong-Hwan Hwang Seungah Cha So-Yeon Jeon Kyu Yun Jang Namsu Kim Chang-Hoon Lee Department of Internal Medicine Jeonbuk National University Medical SchoolJeonju 54907South Korea Department of Pathology Jeonbuk National University Medical SchoolJeonju 54907South Korea Department of Laboratory Medicine Jeonbuk National University Medical SchoolJeonju 54907South Korea

BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare,life-threatening disorder caused by abnormal histiocytes and T cell *** adults,it is predominantly associated with infections,cancers,and autoimmune *** polychondritis(RP),another rare disease,is diagnosed based on symptoms without specific tests,featuring cartilage inflammation characterized by swelling,redness,and pain,rarely inducing *** SUMMARY A 74-year-old woman visited the emergency room with a fever of 38.6℃.Blood tests,cultures,and imaging were performed to evaluate *** showed increased fluorescent antinuclear antibody levels and mild cytopenia,with no other specific *** revealed lymph node enlargement was observed;however,biopsy results were *** re-evaluation of the physical exam,inflammatory signs suggestive of RP were observed in the ears and nose,prompting a tissue biopsy for ***,persistent fever accompanied by cytopenia prompted a bone marrow examination,revealing hemophagocytic *** finding no significant results in blood culture,viral markers,and tissue examination of enlarged lymph nodes,HLH was diagnosed by *** involved methylprednisolone followed by *** two months,bone marrow examination confirmed resolution of hemophagocytosis,with normalization of hyperferritinemia and *** Thorough physical examination enabled diagnosis and treatment of HLH trig gered by RP in patients presenting with fever of unknown origin.

关键词： Hemophagocytic lymphohistiocytosis Relapsing polychondritis autoimmune disease Fever of unknown origin Steroid Case report

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PFA-fixed Hsp6Osp-loaded dendritic cells as a vaccine for the control of mouse experimental allergic encephalomyelitis

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Cellular & Molecular Immunology 2014年第2期11卷 169-174页

作者： Feng Liu Hui Zheng Yuanyuan Qi Xue Wang Jianjun Yang Miaomiao Han Hart Zhang Hong Jiang These authors contributed equally to this work.

We have shown that Hsp6Osp-loaded immature dendritic cells （DC/sp） can protect mice from the induction of experimental allergic encephalomyelitis （EAE） by inducing Qa-l-restricted CD8＋ T regulatory （Treg） cells. The binding half-life between Qa-1 and Hsp6Osp is particularly short and leads to an unstable Qa- l/peptide complex that significantly decreases the efficacy of this vaccination. To prevent Qa-l/Hsp6Osp complex dissociation, we utilized paraformaldehyde （PFA） fixation to stabilize the formation of the Qa-l/Hsp6Osp complex and maximize the function of DC/sp as a vaccine to control autoimmune diseases. Compared with the non-fixed DC/sp, the fixed DC/sp （FDC/sp） showed an enhanced ability to activate Qa-l-restricted Hsp6Osp-specific CD8＋T cells in vitro and prevented EAE in vivo. Importantly, the FDC/sp maintained immune activity following cryopreservation for I week or after storage for 72 h at 4 ~C. These results indicate that PFA fixation can sustain or increase the efficacy of DC/sp by improving the stability of the Qa-l/Hsp6Osp complex on the surface of the DC/sp. In addition, PFA fixation creates a time window for DC/sp storage, transport and application. Our d^tn ~u^p__~t ~ nnt^nti^l clinic^l ..~e nf FDCI^n ~ ~ vnccine fnr the nr^v~ntinn and treatment of autnimm.n~_ di_~_~__

关键词： autoimmune disease dendritic cells Hsp6Osp PFA fixation Qa-l-restricted CD8+ T cells

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The mTOR pathway in the antiphospholipid syndrome

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Medical Review 2023年第6期3卷 511-513页

作者： Lanlan Ji Zhuoli Zhang Andras Perl Department of Rheumatology and Clinical Immunology Peking University First HospitalBeijingChina Department of Rheumatology and Clinical Immunology Peking University First HospitalNo.8Xishiku StreetBeijing 100034China Departments of Medicine Microbiology and ImmunologyBiochemistry and Molecular BiologyDivision of Rheumatology and Clinical ImmunologyState University of New YorkUMUCollege of Medicine750 East Adams StreetSyracuseNY 13210USA

This perspective discussed the available evidence on the involvement of mTOR pathway in antiphospholipid syndrome(APS),from the aspects of endothelial cells,platelets,monocytes and anti-phospholipid antibodies(PLs),wh... 详细信息

关键词： antiphospholipid syndrome mTOR autoimmune disease

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The TGF-β superfamily cytokine Activin-A is induced during autoimmune neuroinflammation and drives pathogenic Th17 cell differentiation

The TGF-β superfamily cytokine Activin-A is induced during ...

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第十四届全国免疫学学术大会

作者： Bing Wu YISONG WAN UNC at Chapel hill 武汉大学

Th17 cells are known to exert pathogenic and non-pathogenic functions. Although the cytokine transforming growth factor b1(TGF-β1) is instrumental for Th17 cell differentiation, it is dispensable for generation of pathogenic Th17 cells. Here, we examined the T cell-intrinsic role of Activin-A, a TGF-β superfamily member closely related to TGF-β1, in pathogenic Th17 cell differentiation. Activin-A expression was increased in individuals with relapsing-remitting multiple sclerosis and in mice with experimental autoimmune encephalomyelitis. Stimulation with interleukin-6 and Activin-A induced a molecular program that mirrored that of pathogenic Th17 cells and was inhibited by blocking Activin-A signaling. Genetic disruption of Activin-A and its receptor ALK4 in T cells impaired pathogenic Th17 cell differentiation in vitro and in vivo. Mechanistically, extracellular-signal-regulated kinase(ERK) phosphorylation, which was essential for pathogenic Th17 cell differentiation, was suppressed by TGF-β1-ALK5 but not Activin-A-ALK4 signaling. Thus, Activin-A drives pathogenic Th17 cell differentiation, implicating the Activin-A-ALK4-ERK axis as a therapeutic target for Th17 cell-related diseases.

关键词： autoimmune disease multiple sclerosis EAE pathogenic Th17 cells Activin-A ALK4 TGF-β1 ALK5 ERK1/2

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Effect of Naringenin on Experimental autoimmune Encephalomyelitis in Mice

Effect of Naringenin on Experimental Autoimmune Encephalomye...

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第十届全国免疫学学术大会

作者： Junpeng Wang Xinli Niu Simin Meydani Wu Dayong Henan University Tufts University

The common therapies for autoimmune and inflammatory diseases have limited efficacy and often come with serious side *** intervention may play a role in developing preventive and therapeutic strategies for autoimmune ***,a flavonoid found in high content in citrus fruits,particularly grapefruits,has been shown to have anti-inflammatory *** present study assessed effect of naringenin on T-cell mediated autoimmunity using the experimental autoimmune encephalomyelitis(EAE),a rodent model for human multiple *** C57BL/6 mice were fed naringenin and then immunized with MOG35-55 to induce *** fed naringenin had delayed onset and attenuated symptoms of EAE,as well as reduced immune cell infiltration and demyelination in spinal cord compared to mice fed the control *** both peripheral lymph nodes and central nervous system,the EAE-evoked upregulation of pro-inflammatory,pathogenic Th1 and Th17 cell populations were reduced by naringenin supplementation while the frequency of antiinflammatory CD4+CD25+Fox P3+regulatory T cells were not ***,we found that decreased Th1 and Th17populations in lymph nodes of naringenin-fed mice were associated with reduced expression of their respective transcription factors,T-bet and RORγ*** addition,pathologic T cell proliferation induced by ex vivo re-stimulation with MOG35-55 was lower in naringenin-fed mice than in the control *** these results suggest that naringenin may have the potential to ameliorate autoimmune disease by favorably modulating autoimmune response.

关键词： Naringenin Th cell subsets autoimmune disease Inflammation

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The Immunopharmaceutical Effects and Mechanisms of Herb Medicine

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Cellular & Molecular Immunology 2008年第1期5卷 23-31页

作者： Chien-Fu Huang Shih-Shen Lin Pao-Hsin Liao Su-Chung Young Chi-Chiang Yang Department of Biological Science and Technology I-Shou UniversityKaoshiung Department of Dentistry Chung Shan Medical University HospitalChung Shan Medical UniversityTaichung Department of Anatomy Chung Shan Medical UniversityTaichung School of Medical Laboratory and Biotechnology Chung Shan Medical UniversityTaichung

In recent years, studies on evaluation of the therapeutic and toxic activity of herbal medicinal products became available and popular. The advances in modern biotechnology have led to discovery of many new active constituents. However, it is a constant challenge to establish the pharmacological basis for efficacy and safety of herbal medicinal products. A better understanding of the effects and bioavailability of phytopharmaceuticals can help in discovering suitable and rational therapies. In this review, we present the bioavailability studies in immune system that has been conducted for some of the more important or widely used phytopharmaceuticals. Furthermore, various new drug targets worthy of using for drug development in immunomodulating herbal medicine area and their regulatory mechanisms are also discussed. Adverse effects, drug interactions, and contraindications are also discussed which show that caution should be exercised when combining phytopharmaceuticals with chemically derived pharmaceutical components. Cellular ＆ Molecular Immunology.

关键词： phytomedicine herbal medicine alternative medicine T-lymphocyte autoimmune disease

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Generation and Regulation of CD8^+ Regulatory T Cells

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Cellular & Molecular Immunology 2008年第6期5卷 401-406页

作者： Linrong Lu Harvey Cantor Department of Cancer Immunology ＆ AIDS Dana-Farber Cancer Instituteand Department of Pathology Harvard Medical School Boston MA 02115USA Institute of Immunology Zhejiang University School of MedicineHangzhou China Corresponding to： Dr. Harvey Cantor Department of Cancer Immunology ＆ AIDS Dana-Farber Cancer Institute and Department of Pathology Harvard Medical School 44 Binney Street Boston MA 02115 USA

Research into the suppressive activity of CD4＋FoxP3＋ T regulatory cells （Treg） has defined a sublineage of CD4＋ cells that contribute to self-tolerance and resistance to autoimmune disease. Much less attention has been given to the potential contribution of regulatory sublineages of CD8＋ cells. Analysis of a small fraction of CD8＋ cells that target autoreactive CD4＋ cells through recognition of the MHC class Ib molecule Qa-1 in mouse and HLA-E in human has revitalized interest in CD8＋ Treg. Here we summarize recent progress and future directions of research into the role of this CD8＋ sublineage in resistance to autoimmune disease. Cellular ＆ Molecular Immunology. 2008; 5（6）：401-406.

关键词： CD8+ regulatory T cell self-tolerance autoimmune disease EAE Qa-1 NKG2A

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Variants in the Promoter Region of HLA-DQA1 were Associated with Idiopathic Membranous Nephropathy in a Chinese Han Population

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Chinese Medical Journal 2017年第14期130卷 1677-1682页

作者： Xiao-Song Qin Jian-Hua Liu Guan-Ting Lyu Meng-Le Peng Fu-Ning Yang Dong-Chun Qin Yong-Zhe Li Yong Liu Department of Medical Laboratory Shengjing Hospital of China Medical University Shenyang Liaoning 110011 China Department of Blood Transfusion Tangdu Hospital The Fourth Military Medical University Xi&#39 an Shaanxi 710038 China Department of Medical Laboratory The First Affiliated Hospital of Zhengzhou University Zhengzhou Henan 450052 China Department of Rheumatology and Clinical immunology Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Key Laboratory of Rheumatology and Clinical Immunology Ministry of Education Beijing 100005 China

Background：Idiopathic membranous nephropathy （IMN） is an autoimmune disease and the leading cause of adult nephritic ***-DQA1 had been identified to be associated with IMN in Europeans and the result was replicated in Chinese Han *** this study,six single nucleotide polymorphisms （SNPs） in the promoter ofHLA-DQA I and other two SNPs with IgA nephropathy were included for the association ***：The SNPs were genotyped in 509 patients and 601 controls by the MassArray *** quantification ofanti-phospholipase A2 receptor （PLA2R） antibodies in sera of IMN patients was performed by anti-PLA2R ELISA （lgG） ***：After analysis,four SNPs were significantly associated with IMN,with rs2187668 and rs28383345 as the top two signals （P =8.42×10-4 and 2.48× 10-5,respectively）.Even under dominant model,the two SNPs were still significantly associated with IMN （P =3.50×10-3 for rs28383345 and P =6.55×10-5 for rs2187668）.After conditional study with rs2187668,rs28383345 was the only variant significantly correlated with IMN after Bonferroni correction （P =0.016）.The minor alleles of the two SNPs were also mutually exclusive in our *** indicated that the two SNPs were independently associated with IMN in Chinese Han *** of anti-PLA2R autoantibodies were correlated with the genotypes of the two SNPs,but not significantly （P〉0.05）.Conclusions：Our results revealed that a novel independent variant in the promoter of HLA-DQA I was associated with I MN in Chinese Han *** locus possessed regulatory role according to the data of *** exact role of the SNPs on the expression of HLAoDQA1 needs further investigation.

关键词： autoimmune disease HLA-DQA1 Idiopathic Membranous Nephropathy Promoter Chinese

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Role of Leptin in Immunity

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Cellular & Molecular Immunology 2007年第1期4卷 1-13页

作者： Queenie Lai Kwan Lam Department of Pathology and Center of Infection and Immunology The University of Hong Kong

Leptin, a protein hormone produced by the adipocytes, has long been recognized to regulate metabolism, neuroendorine and other physiological functions. Early findings of increased leptin production during infection and inflammation and dysregulated immune response in leptin signaling-deficient mice provide strong evidence for the involvement of leptin in the immune responses. Recent data have established the regulatory function for leptin in immunity similar to the function of a pro-inflammatory cytokine, while gene-targeting studies also demonstrated an essential role of leptin in regulating hematopoiesis and lymphopoiesis. Moreover, there has been increasing evidence that leptin is involved in the pathogenesis of various autoimmune diseases. This review discusses recent advances in understanding the role of leptin in immunity and leptin-signaling pathways involved in modulating immune homeostasis and autoimmune pathogenesis. Cellular ＆ Molecular Immunology. 2007;4（1）：1-13.

关键词： leptin leptin signaling pro-inflammatory cytokine immune response autoimmune disease

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Detection of anti-aquaporin-4 autoantibodies in the sera of Chinese neuromyelitis optica patients

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Neural Regeneration Research 2013年第8期8卷 708-713页

作者： Miao Li Weiheng Su Jie Wang Francesco Pisani Antonio Frigeri Tonghui Ma Membrane Channel Research Laboratory Northeast Normal University Department of Neurosurgery China-Japan Union HospitalJilin University National Engineering Laboratory for AIDS Vaccine College of Life ScienceJilin University Department of General and Environmental Physiology and Centre of Excellence in Comparative Genomics (CEGBA) University of Bari

In this study, we recruited 10 neuromyelitis optica patients, two multiple sclerosis patients and two myelitis patients. Chinese hamster lung fibroblast （V79） cells transfected with a human aquaporin-4-mCherry fusion protein gene were used to detect anti-aquaporin-4 antibody in neuromyelitis optica patient sera by immunofluorescence. Anti-aquaporin-4 autoantibody was stably detected by immunofluorescence in neuromyelitis optica patient sera exclusively. The sensitivity of the assay for neuromyelitis optica was 90% and the specificity for neuromyelitis optica was 100%. The anti-aquaporin-4 antibody titers in sera were tested with serial dilutions until the signal disappeared. A positive correlation was detected between Expanded Disability Status Scale scores and serum anti-aquaporin-4 antibody titers. The anti-aquaporin-4 antibody assay is highly sensitive and specific in the sera of Chinese neuromyelitis optica patients. Detection of aquaporin-4 autoantibody is important for the diagnosis and treatment of neuromyelitis optica.

关键词： neural regeneration neurodegenerative diseases neuromyelitis optica multiple sclerosis aquaporin-4 autoimmune disease autoantibody immunofluorescence Expanded Disability StatusScale grants-supported paper photographs-containing paper neuroregeneration

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