ISSN : 2146-3123
E-ISSN : 2146-3131

Autoinflammatory Diseases in Childhood
Mehmet Yıldız1, Fatih Haşlak1, Amra Adrovic1, Kenan Barut1, Özgür Kasapçopur1
1Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa Cerrahpaşa School of Medicine, İstanbul, Turkey
DOI : 10.4274/balkanmedj.galenos.2020.2020.4.82

Autoinflammatory diseases are characterized by recurrent fever attacks and clinical findings that arise from impaired natural immunity and spread to a wide variety of organ systems. The concept of autoinflammatory disease emerged after the definition of familial Mediterranean fever (FMF) and Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS). Firstly, it is thought that this new group differs from the standard concept of autoimmune diseases, which is relatively better known in terms of basic features such as defects in innate immunity and the absence of antibodies. We have come a long way in understanding the genetic and pathogenetic mechanisms of this relatively new disease group in the past twenty years since they were first diagnosed. This growing understanding leads to some changes in the concept of autoinflammatory diseases. Recently, the definition of the autoinflammatory disease includes a wide range of diseases with different clinical features, mainly accompanied by changes in innate immune and rarely in humoral immunity. Owing to recent developments in molecular sciences and genetics, the spectrum of autoinflammatory diseases is rapidly expanding. In this review, clinical features, classification criteria, treatment options and long-term prognoses of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) syndrome, and other common autoinflammatory diseases are discussed in the light of current literature.

Keywords : Autoinflammatory diseases, CAPS, childhood, familial Mediterranean fever, FMF, TRAPS

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