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A Glance at History and Future Perspectives of Childhood Autoinflammatory Disorders

Adem Polat, Erkan Demirkaya, Gokalp Basbozkurt, Marco Gattorno, Seza Ozen; FMF Arthritis Vasculitis and Orphan disease Research in Paediatric Rheumatology (FAVOR).

Cited by (4)

Abstract
Autoinflammatory diseases are described as recurrent and self limiting disorders of the innate immune system and thought to be caused by a defect in regulating inflammatory response. First description of the term autoinflammatory was made by Mc Dermott et al. Since then different syndromes presenting with periodic attacks have been described. The common inheritance pattern of them were monogenic. Some other disorders were also gathered under this headline, considering their clinical presentation rather than molecular basis or having a Mendelian pattern of inheritance. Our aim with this review article is to take a glance at history, clinical manifestations, diagnosis, current and new approaches and future perspectives for childhood autoinflammatory diseases for pediatricians and pediatric rheumatologists.

Key words: Autoinflammatory diseases, HIDS, FMF, cryopyrinopathies



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