Immunodeficiencies and Autoimmune Diseases (a Scientific Colloquium)

免疫缺陷和自身免疫性疾病（科学研讨会）

• 批准号: 7752767
• 负责人: Noel R. Rose
• 金额: $ 0.7万
• 依托单位: AMERICAN AUTOIMMUNE RELATED DIS ASSOC
• 依托单位国家: 美国
• 财政年份: 2009
• 资助国家: 美国
• 起止时间: 2009-09-28 至 2010-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7752767
• 关键词: Address; Adopted; American; Animals; Area; Autoimmune Diseases; Autoimmune Process; Autoimmunity; B-Lymphocytes; Cells; Collaborations; Common Variable Immunodeficiency; Communicable Diseases; Curiosities; Defect; Development; Discipline; Disease; Disease Association; Funding; Genetic; Goals; Hematologist; Hemolytic Anemia; Human; Immune; Immune response; Immune system; Immunologic Deficiency Syndromes; Immunologist; Knowledge; Mediator of activation protein; Molecular; Mutation; Patients; Pernicious Anemia; Primary biliary cirrhosis; Regulation; Reporting; Research; Research Personnel; Rheumatoid Arthritis; Risk; Role; Scientist; Sicca Syndrome; Specialist; Systemic Lupus Erythematosus; T-Lymphocyte; Telefacsimile; Telephone; Thrombocytopenia; Time; Translational Research; Treatment Failure; United States National Institutes of Health; Work; base; immunoregulation; interdisciplinary approach; medical specialties; meetings; response; rheumatologist

DESCRIPTION (provided by applicant): Although autoimmunity is as an over response of the immune system and immunodeficiency is an under response, immunodeficiency diseases and autoimmune diseases are not mutually exclusive since both represent profound dysregulation of the immune response. Immunodeficiencies are associated with impaired function of one of the cells or soluble mediators the immune system. For example, approximately 20 % of patients with common variable immunodeficiency (CVID), a B cell defect, develop an autoimmune condition such as idiopathic thrombocytopenia (ITP), hemolytic anemia, rheumatoid arthritis, sicca syndrome, primary biliary cirrhosis, and pernicious anemia. Systemic lupus erythematosis (SLE) may also co-occur with CVID, but the development of SLE may precede or follow the development of CIVD, illustrating the two way relationship between autoimmunity and immunodeficiency based on fundamental defects in immunoregulation deficiency. New opportunities to study genetic mechanisms that help to explain the connection between T cell deficiencies in autoimmune disease are rapidly emerging. In addition to the human disorders some of these genetic defects have been reproduced in experimental animals where they are providing the unique opportunity to study the molecular mechanisms underlying in both autoimmunity and immunodeficiency. We believe that these exciting opportunities to explore the relationship between immunodeficiency diseases and autoimmune diseases makes a meeting on this topic particularly timely. The goal of the colloquium is to identify critical research areas that represent unmet needs or are presently under explored. The meeting will bring together expert investigators in different specialties to identify the basic issues that may explain the co-occurrence of immunodeficiency and autoimmunity. At the same time new opportunities for translational research will arise to benefit immunodeficiency patients who may develop autoimmune disease or autoimmune disease patients at risk for immunodeficiency. By bringing together geneticists, hematologists, infectious disease specialists, and immunologists together with molecular scientists we intend to challenge and expand the way we presently think about the regulation of immune responses.

描述（由申请人提供）：尽管自身免疫性是免疫系统的过度反应，并且免疫缺陷是一种反应，但免疫缺陷疾病和自身免疫性疾病并非相互排斥，因为两者都代表了免疫反应的深刻失调。免疫缺陷与其中一个细胞或可溶性介质的功能受损有关。例如，大约20％的患有常见可变免疫缺陷的患者（CVID）是B细胞缺陷，患有自身免疫性疾病，例如特分病血栓细胞减少症（ITP），溶血性贫血，类风湿关节炎，西卡综合征，Sisca综合征，主要胆汁性肝硬化。全身性红斑狼疮（SLE）也可能与CVID共发生，但SLE的发展可能是在CIVD之前或遵循CIVD的发展，这说明了基于免疫调节缺乏症的基本缺陷，自身免疫性和免疫缺陷之间的两种关系关系。研究遗传机制的新机会，有助于解释自身免疫性疾病中T细胞缺陷之间的联系。除了人类疾病外，这些遗传缺陷中的一些已经在实验动物中再现了，它们提供了研究自身免疫和免疫缺陷的独特机会来研究分子机制。我们认为，这些令人兴奋的机会探索免疫缺陷疾病与自身免疫性疾病之间的关系使有关该主题的会议特别及时。座谈会的目的是确定代表未满足需求或目前正在探索的关键研究领域。会议将汇集不同专业的专家调查员，以确定可能解释免疫缺陷和自身免疫性的同时出现的基本问题。同时，将出现转化研究的新机会，以使可能患有自身免疫性疾病或自身免疫性疾病患者受到免疫缺陷风险的患者。通过将遗传学家，血液学家，传染病专家和免疫学家汇集在一起​​，以及分子科学家，我们打算挑战和扩展我们目前对免疫反应调节的看法。