Autoinflammatory novelties: from pathogenic mechanisms to clinical and therapeutic implications: Volume II

Many recent novelties have been highlighted in systemic autoinflammatory diseases (SAIDs): rare disorders mainly activated by an aberrant activation of the innate immune system which may provoke abrupt episodes of organ-specific and systemic inflammation. Interestingly, multiple lines of evidence also report the clinical relevance of these conditions in adult patients, not only in childhood. Prompted by the discovery of VEXAS syndrome, a prototype of adult autoinflammatory disease has been proposed in paving the way for further studies in this field.The interaction of genetic background and triggers in leading to the deregulated immune response of SAIDs in adults is not fully understood yet. In some cases, the genetic cause is recognized whereas in other conditions polygenic and multifactorial steps are hypothesized. In addition, in SAIDs, the persistent activation of inflammasomes needs to be furtherly clarified together with the associated inflammatory cytokine cascades. From a clinical point of view, SAIDs may be characterized by a heterogeneous clinical picture in addition to the classic hallmark of fever. In fact, from moderate severity to life-threatening conditions may be recognized making more difficult the management of these patients. In fact, mostly in adults, the missing of specific diagnostic criteria leads to a delayed diagnosis. This situation is furtherly worsened by the lack of specific biomarkers in guiding the diagnosis and prognosis.Finally, it remains to be fully established the most appropriate therapeutic strategy in order to achieve and maintain the clinical response as well as to prevent the occurrence of life-threatening evolution and damage.In this article collection, the preparation of original research articles is encouraged but also brief research reports, reviews, general commentaries, systematic literature reviews, systematic literature reviews and meta-analysis are welcome.Prof. Paola Cipriani declares participation in a clinical study with Janssen and participation in congresses with UCB. All other Topic Editors declare no conflict of interest. Topic editors: Ilenia Di Cola, Rim Bourguiba, Paola Cipriani Source: https://www.frontiersin.org/research-topics/81589/autoinflammatory-novelties-from-pathogenic-mechanisms-to-clinical-and-therapeutic-implications-volume-ii