Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 15 6 2017 12 29 430 444 EN Gholamreza Azizi Department 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, Iran Shahin Khadem Azarian Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Sepideh Nazeri Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Ali Mosayebian Department of Immunology, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran Saleh Ghiasy Research Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran Ghazal Sadri Research Centre for Immunodeficiencies, Pediatrics Centre of Excellence, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran Ali Mohebi Growth and Development Research Centre, Children's Medical Centre, Tehran, University of Medical Sciences, Tehran, Iran Nikoo Hossein Khan Nazer Department of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sanaz Afraei Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran Abbas Mirshafiey Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran 2016 03 05 2016 09 05 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.  https://ijaai.tums.ac.ir/index.php/ijaai/article/view/837 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/837/682