Autoinflammatory syndromes.

There has been an expansion of the autoinflammatory syndromes due to the discovery of new diseases related to mutations in genes regulating the innate immune system and the knowledge gained from these diseases as applied to more common nongenetic inflammatory conditions. Autoinflammatory syndromes are characterized by unprovoked (or triggered by minor events) recurrent episodes of systemic inflammation involving various body systems, which are often accompanied by fever. Inflammation is mediated by polymorphonuclear and macrophage cells through cytokines, particularly interleukin-1. This article reviews the clinical approach to patients with suspected autoinflammatory syndromes, several of the main and new (mostly genetics) syndromes, advances in treatment, and prognosis.

Hashkes PJ ，Toker O 《-》

[Hereditary periodical fever syndromes].

Herlin T ，Sørensen N ，Veirum J 《-》

Periodic Fever syndromes.

Wurster VM ，Carlucci JG ，Edwards KM 《PEDIATRIC ANNALS》

Monogenic Periodic Fever Syndromes: Treatment Options for the Pediatric Patient.

Ozen S ，Demir S 《-》

The fresco of autoinflammatory diseases from the pediatric perspective.

Autoinflammatory diseases are genetic or acquired clinical entities globally caused by the aberrant release of the proinflammatory cytokine interleukin-1 and mostly characterized by recurrent spontaneous inflammatory events which do not produce antigen-specific T cells or autoantibodies. Within the past decade, the list of autoinflammatory diseases has included cryopyrin-associated periodic syndromes, familial Mediterranean fever, mevalonate kinase deficiency, tumor necrosis factor receptor-associated periodic syndrome, hereditary pyogenic disorders, pediatric granulomatous autoinflammatory diseases, idiopathic febrile syndromes, complement dysregulation syndromes and Behçet's disease. Most of these conditions interact with the inflammasomes, intracellular molecular complexes coordinating the phylogenetically ancient response of the innate immune system. The pathogenetic mechanisms of these diseases have shown the evidence of disrupted interleukin-1 signaling for most of them and allowed to locate interleukin-1 as an attractive therapeutic target. The whole fresco of autoinflammatory diseases in pediatrics will be discussed in this review with the aim of establishing both diagnostic clues and treatments for each condition.

Rigante D 《-》