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....
| Publicado en: | Iranian Journal of Allergy, Asthma & Immunology Vol. 15; no. 6; pp. 430 - 445 |
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| Autores principales: | , , , , , , , , , , , , |
| Formato: | review Journal Article |
| Publicado: |
Tehran University of Medical Sciences
Dec2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=120790617&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 120790617 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 17351502 87XG jtl: Iranian Journal of Allergy, Asthma & Immunology issn: 17351502 maglogo: N pubinfo: dt: Dec2016 vid: 15 iid: 6 pid: 21783 pub: Tehran University of Medical Sciences artinfo: ui: 120790617 120790617 NLM28129677 120790617 NLM28129677 120790617 ppf: 430 ppct: 15 formats: tig: atl: Monogenic Auto-inflammatory Syndromes: A Review of the Literature. aug: au: Azizi, Gholamreza Azarian, Shahin Khadem Nazeri, Sepideh Mosayebian, Ali Ghiasy, Saleh Sadri, Ghazal Mohebbi, Ali Nazer, Nikoo Hossein Khan Afraei, Sanaz Mirshafiey, Abbas Khadem Azarian, Shahin Mohebi, Ali Khan Nazer, Nikoo Hossein affil: Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran sug: subj: Hereditary Autoinflammatory Diseases Immunology Macrophages Immunology Interleukin 1 Immunology Monocytes Immunology Synovitis Arthritis, Infectious Immunology Cryopyrin-Associated Periodic Syndromes Drug Therapy Antirheumatic Agents Therapeutic Use Acne Vulgaris Drug Therapy Pyoderma Gangrenosum Drug Therapy Proteins Therapeutic Use Autoimmune Diseases Immunology Arthritis Immunology Hereditary Autoinflammatory Diseases Drug Therapy Mevalonate Kinase Deficiency Immunity Immunology Synovitis Drug Therapy Synovitis Immunology Arthritis Drug Therapy Cryopyrin-Associated Periodic Syndromes Immunology Pyoderma Gangrenosum Immunology Mevalonate Kinase Deficiency Drug Therapy Hereditary Autoinflammatory Diseases Arthritis Uveitis Immunology Uveitis Drug Therapy Mevalonate Kinase Deficiency Immunology Arthritis, Infectious Drug Therapy Autoimmune Diseases Pyoderma Gangrenosum Arthritis, Infectious Uveitis Acne Vulgaris Immunology Cryopyrin-Associated Periodic Syndromes Autoimmune Diseases Drug Therapy Acne Vulgaris ab: 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. pubtype: Academic Journal doctype: review Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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