Cytokine signatures in hereditary fever syndromes (HFS)

• Published in: Cytokine & growth factor reviews 2017-02, Vol.33, p.19-34
• Main Authors: Ibrahim, José Noel, Jéru, Isabelle, Lecron, Jean-Claude, Medlej-Hashim, Myrna
• Format: Article
• Language: English
• Subjects: Advanced Basic Science; Cytokines; ex vivo; Hereditary fever syndromes; PBMC; Serum
• ISSN: 1359-6101
• DOI: 10.1016/j.cytogfr.2016.11.001

Highlights • The current review reports a number of advances in the field of inflammatory/immunological mechanisms underlying HFS, and presents all available data regarding the cytokines’ signatures in HFS at the transcriptional, serum and ex vivo levels. • It also integrates recent data in an updated classification of the different HFS in which CAPS, NLRP12AD and FMF were now classified as inflammasomopathies, HIDS/MKD as metabolic dysregulation leading to IL-1β secretion, and TRAPS and the last HFS identified to date, TRAPS11, as protein misfolding disorders. • Cytokine signatures obtained in patients’ cells cultured ex vivo are much more convincing than results obtained at the transcriptional levels or in sera. • Elevated levels of acute phase reactants and pro-inflammatory cytokines in remission periods confirm the involvement of the cytokine network in the inflammatory process and provide evidence for the ongoing subclinical inflammation during asymptomatic periods of HFS. • The marked increase of IL-1β production by LPS-stimulated PBMCs observed in CAPS and NLRP12AD, but also in FMF and MKD, is a hallmark of these disorders.