Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System
Inborn errors of immunity (IEI) are immune system disorders caused by genetic mutations, often presenting with varying degrees of infection, immune dysregulation, lymphoproliferation, and tumor susceptibility. Initially, IEIs were typically diagnosed in patients with recurrent and unusual infections...
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| Format: | Article |
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Editorial Office of Journal of Rare Diseases
2024-10-01
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| Series: | 罕见病研究 |
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| Online Access: | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2024.04.001 |
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| author | ZHOU Yu SONG Hongmei |
| author_facet | ZHOU Yu SONG Hongmei |
| author_sort | ZHOU Yu |
| collection | DOAJ |
| description | Inborn errors of immunity (IEI) are immune system disorders caused by genetic mutations, often presenting with varying degrees of infection, immune dysregulation, lymphoproliferation, and tumor susceptibility. Initially, IEIs were typically diagnosed in patients with recurrent and unusual infections. However increasing research has shown that noninfectious manifestations can also be the initial or even primary presentation of IEI. Over the past ten years, more and more IEIs associated with autoinflammatory symptoms have been identified. Although these diseases are rare, relevant research suggests that immune deficiency and autoinflammation are not opposing conditions but rather interconnected aspects of the immune system, influencing each other in a complementary and inseparable manner. This article reviews the mechanisms involved in IEI with autoinflammation, and proposes some clues for identifying IEI manifested as autoinflammation. It also summarizes the current progress in the diagnosis and treatment of IEI manifested as autoinflammation, and presents prospects for future research on IEI. |
| format | Article |
| id | doaj-art-9f8bd23091214eaabb2955e6e4215052 |
| institution | Kabale University |
| issn | 2097-0501 |
| language | zho |
| publishDate | 2024-10-01 |
| publisher | Editorial Office of Journal of Rare Diseases |
| record_format | Article |
| series | 罕见病研究 |
| spelling | doaj-art-9f8bd23091214eaabb2955e6e42150522025-01-08T05:17:05ZzhoEditorial Office of Journal of Rare Diseases罕见病研究2097-05012024-10-013441141510.12376/j.issn.2097-0501.2024.04.001Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune SystemZHOU Yu0SONG Hongmei1Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaInborn errors of immunity (IEI) are immune system disorders caused by genetic mutations, often presenting with varying degrees of infection, immune dysregulation, lymphoproliferation, and tumor susceptibility. Initially, IEIs were typically diagnosed in patients with recurrent and unusual infections. However increasing research has shown that noninfectious manifestations can also be the initial or even primary presentation of IEI. Over the past ten years, more and more IEIs associated with autoinflammatory symptoms have been identified. Although these diseases are rare, relevant research suggests that immune deficiency and autoinflammation are not opposing conditions but rather interconnected aspects of the immune system, influencing each other in a complementary and inseparable manner. This article reviews the mechanisms involved in IEI with autoinflammation, and proposes some clues for identifying IEI manifested as autoinflammation. It also summarizes the current progress in the diagnosis and treatment of IEI manifested as autoinflammation, and presents prospects for future research on IEI.https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2024.04.001inborn errors of immunityautoinflammatoryimmune dysfunction |
| spellingShingle | ZHOU Yu SONG Hongmei Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System 罕见病研究 inborn errors of immunity autoinflammatory immune dysfunction |
| title | Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System |
| title_full | Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System |
| title_fullStr | Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System |
| title_full_unstemmed | Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System |
| title_short | Immune Deficiency and Autoinflammation, the " Yin" and " Yang" of the Immune System |
| title_sort | immune deficiency and autoinflammation the yin and yang of the immune system |
| topic | inborn errors of immunity autoinflammatory immune dysfunction |
| url | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2024.04.001 |
| work_keys_str_mv | AT zhouyu immunedeficiencyandautoinflammationtheyinandyangoftheimmunesystem AT songhongmei immunedeficiencyandautoinflammationtheyinandyangoftheimmunesystem |