Author/Authors :
Azizi، Gholamreza نويسنده Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran. Azizi, Gholamreza , Khadem Azarian، Shahin نويسنده Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Khadem Azarian, Shahin , Nazeri، Sepideh نويسنده Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Nazeri, Sepideh , Mosayebian، Ali نويسنده Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Mosayebian, Ali , Ghiasy، Saleh نويسنده Research Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Childrens Medical Centre, Tehran University of Medical Sciences, Tehran, Iran Ghiasy, Saleh , Sadri، Ghazal نويسنده Research Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Childrens Medical Centre, Tehran University of Medical Sciences, Tehran, Iran Sadri, Ghazal , Mohebi، Ali نويسنده Growth and Development Research Centre, Childrens Medical Centre, Tehran, University of Medical Sciences, Tehran, Iran Mohebi, Ali , Khannazer، Nikoo نويسنده Department of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran Khannazer, Nikoo , Afraei، Sanaz نويسنده Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Afraei, Sanaz , Mirshafiey، Abbas نويسنده ,
Abstract :
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.
