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Periodic fever syndrome, autoinflammatory diseases

Publication at First Faculty of Medicine |
2023

Abstract

Autoinflammatory diseases (AID) include a diverse group of diseases defined by dysregulation of the innate immune system. Clinical manifestations of sterile inflammation include usually episodic fever with variable presence of organ involvement.

A more detailed understanding of the etiopathogenetic background of AID opens new perspectives for targeted therapies. Disease activity control and prevention of organ damage are the main treatment goals.

Multidisciplinary management of AID should be concentrated in specialised centers. The best known group of AIDs,periodic fever syndromes,include Familial Mediterranean fever (FMF), Mevalonate kinase deficiency (MKD), Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), Cryopyrin-associated periodic syndromes (CAPS) and Periodic fever with aphthae, pharyngitis and cervical adenitis (PFAPA ).

The article provides general information about the group of autoinflammatory diseases and the most common types of periodic fevers.