Monogenic Auto-inflammatory Syndromes: A Review of the Literature
Auto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto-antibodies or auto-reactive T cells, and an inborn error of innate immunity....
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2017-12-01
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| Series: | Iranian Journal of Allergy, Asthma and Immunology |
| Subjects: | |
| Online Access: | https://ijaai.tums.ac.ir/index.php/ijaai/article/view/837 |
| _version_ | 1811277674489839616 |
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| author | Gholamreza Azizi Shahin Khadem Azarian Sepideh Nazeri Ali Mosayebian Saleh Ghiasy Ghazal Sadri Ali Mohebi Nikoo Hossein Khan Nazer Sanaz Afraei Abbas Mirshafiey |
| author_facet | Gholamreza Azizi Shahin Khadem Azarian Sepideh Nazeri Ali Mosayebian Saleh Ghiasy Ghazal Sadri Ali Mohebi Nikoo Hossein Khan Nazer Sanaz Afraei Abbas Mirshafiey |
| author_sort | Gholamreza Azizi |
| collection | DOAJ |
| description | Auto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto-antibodies or auto-reactive T cells, and an inborn error of innate immunity. A narrative literature review was carried out of studies related to auto-inflammatory syndromes to discuss the pathogenesis and clinical manifestation of these syndromes. This review showed that the main monogenic auto-inflammatory syndromes are familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), Blau syndrome, TNF receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA). The data suggest that correct diagnosis and treatment of monogenic auto-inflammatory diseases relies on the physicians’ awareness. Therefore, understanding of the underlying pathogenic mechanisms of auto-inflammatory syndromes, and especially the fact that these disorders are mediated by IL-1 secretion stimulated by monocytes and macrophages, facilitated significant progress in patient management. |
| first_indexed | 2024-04-13T00:21:01Z |
| format | Article |
| id | doaj.art-3e997c145d924baca8100cd955999af2 |
| institution | Directory Open Access Journal |
| issn | 1735-1502 1735-5249 |
| language | English |
| last_indexed | 2024-04-13T00:21:01Z |
| publishDate | 2017-12-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Iranian Journal of Allergy, Asthma and Immunology |
| spelling | doaj.art-3e997c145d924baca8100cd955999af22022-12-22T03:10:46ZengTehran University of Medical SciencesIranian Journal of Allergy, Asthma and Immunology1735-15021735-52492017-12-01156652Monogenic Auto-inflammatory Syndromes: A Review of the LiteratureGholamreza Azizi0Shahin Khadem Azarian1Sepideh Nazeri2Ali Mosayebian3Saleh Ghiasy4Ghazal Sadri5Ali Mohebi6Nikoo Hossein Khan Nazer7Sanaz Afraei8Abbas Mirshafiey9Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran AND Research Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Medicine, Mazandaran University of Medical Sciences, Sari, IranResearch Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, IranResearch Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, IranGrowth and Development Research Centre, Children's Medical Centre, Tehran, University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, IranDepartment of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, IranAuto-inflammatory syndromes are a new group of distinct hereditable disorders characterized by episodes of seemingly unprovoked inflammation (most commonly in skin, joints, gut, and eye), the absence of a high titer of auto-antibodies or auto-reactive T cells, and an inborn error of innate immunity. A narrative literature review was carried out of studies related to auto-inflammatory syndromes to discuss the pathogenesis and clinical manifestation of these syndromes. This review showed that the main monogenic auto-inflammatory syndromes are familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), Blau syndrome, TNF receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA). The data suggest that correct diagnosis and treatment of monogenic auto-inflammatory diseases relies on the physicians’ awareness. Therefore, understanding of the underlying pathogenic mechanisms of auto-inflammatory syndromes, and especially the fact that these disorders are mediated by IL-1 secretion stimulated by monocytes and macrophages, facilitated significant progress in patient management.https://ijaai.tums.ac.ir/index.php/ijaai/article/view/837Auto-inflammatory syndromeFeverInflammationInnate immune response |
| spellingShingle | Gholamreza Azizi Shahin Khadem Azarian Sepideh Nazeri Ali Mosayebian Saleh Ghiasy Ghazal Sadri Ali Mohebi Nikoo Hossein Khan Nazer Sanaz Afraei Abbas Mirshafiey Monogenic Auto-inflammatory Syndromes: A Review of the Literature Iranian Journal of Allergy, Asthma and Immunology Auto-inflammatory syndrome Fever Inflammation Innate immune response |
| title | Monogenic Auto-inflammatory Syndromes: A Review of the Literature |
| title_full | Monogenic Auto-inflammatory Syndromes: A Review of the Literature |
| title_fullStr | Monogenic Auto-inflammatory Syndromes: A Review of the Literature |
| title_full_unstemmed | Monogenic Auto-inflammatory Syndromes: A Review of the Literature |
| title_short | Monogenic Auto-inflammatory Syndromes: A Review of the Literature |
| title_sort | monogenic auto inflammatory syndromes a review of the literature |
| topic | Auto-inflammatory syndrome Fever Inflammation Innate immune response |
| url | https://ijaai.tums.ac.ir/index.php/ijaai/article/view/837 |
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