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- Title
Monogenic Auto-inflammatory Syndromes: A Review of the Literature.
- Authors
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
- 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.
- Subjects
AUTOANTIBODIES; INFLAMMATION; INFLAMMATION treatment; FAMILIAL Mediterranean fever; INTERLEUKIN-1; DIAGNOSIS; DRUG therapy for arthritis; THERAPEUTIC use of proteins; ANTIRHEUMATIC agents; ACNE; ARTHRITIS; AUTOIMMUNE diseases; GENETIC disorders; IMMUNITY; INFECTIOUS arthritis; MACROPHAGES; MONOCYTES; UVEITIS; SYNOVITIS; PYODERMA gangrenosum; MEVALONATE kinase deficiency; CRYOPYRIN-associated periodic syndromes
- Publication
Iranian Journal of Allergy, Asthma & Immunology, 2016, Vol 15, Issue 6, p430
- ISSN
1735-1502
- Publication type
journal article