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）：虽然自身免疫是免疫系统的过度反应，而免疫缺陷是反应不足，但免疫缺陷疾病和自身免疫疾病并不相互排斥，因为两者都代表了免疫反应的严重失调。免疫缺陷与免疫系统细胞或可溶性介质之一的功能受损有关。例如，大约 20% 的常见变异型免疫缺陷 (CVID)（一种 B 细胞缺陷）患者会出现自身免疫性疾病，如特发性血小板减少症 (ITP)、溶血性贫血、类风湿性关节炎、干燥综合征、原发性胆汁性肝硬化和恶性贫血。系统性红斑狼疮（SLE）也可能与 CVID 同时发生，但 SLE 的发展可能先于或晚于 CIVD 的发展，这说明了基于免疫调节缺陷的基本缺陷，自身免疫和免疫缺陷之间的双向关系。研究遗传机制的新机会正在迅速出现，这些机制有助于解释自身免疫性疾病中 T 细胞缺陷之间的联系。除了人类疾病之外，其中一些遗传缺陷已在实验动物中重现，它们为研究自身免疫和免疫缺陷的分子机制提供了独特的机会。我们相信，这些探索免疫缺陷疾病与自身免疫疾病之间关系的令人兴奋的机会使得关于这一主题的会议显得尤为及时。研讨会的目标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研究人员，以确定可以解释免疫缺陷和自身免疫同时发生的基本问题。与此同时，转化研究的新机会也将出现，使可能患有自身免疫性疾病的免疫缺陷患者或有免疫缺陷风险的自身免疫性疾病患者受益。通过将遗传学家、血液学家、传染病专家、免疫学家和分子科学家聚集在一起，我们打算挑战和扩展我们目前对免疫反应调节的思考方式。