{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T03:15:16Z","timestamp":1787022916536,"version":"build-2736575974"},"reference-count":74,"publisher":"American Academy of Pediatrics (AAP)","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,1]]},"abstract":"<jats:p>Children with intermittent fevers present to pediatricians and other primary care child health providers for evaluation. Most patients will have self-limited, benign infectious illnesses. However, the possibility of a periodic fever syndrome should be considered if febrile episodes become recurrent over an extended period and are associated with particular signs and symptoms during each attack. This review discusses the current conceptualization of autoinflammatory diseases with specific focus and detail on familial Mediterranean fever; tumor necrosis factor receptor\u2013associated periodic syndrome; mevalonate kinase deficiency; NLRP3-associated autoinflammatory disease; and periodic fever, aphthous stomatitis, pharyngitis, and adenitis. The genetic mutations associated with these clinical entities are identified, along with the historical nomenclature that predates the current pathogenetic understanding of these diseases. The episodic signs and symptoms seen across these periodic fever syndromes can be overlapping, but there are some distinguishing features that can be useful, and these are described. The disease course and potential complications, particularly amyloidosis, which is a variable risk in these conditions and a potential source of significant morbidity and mortality, are addressed. Treatment strategies are outlined, highlighting the advances in therapy that have resulted from the advent of proinflammatory cytokine\u2013targeting biological agents.<\/jats:p>","DOI":"10.1542\/pir.2022-005635","type":"journal-article","created":{"date-parts":[[2023,8,31]],"date-time":"2023-08-31T23:07:09Z","timestamp":1693523229000},"page":"481-490","source":"Crossref","is-referenced-by-count":10,"title":["Autoinflammatory Diseases\/Periodic Fevers"],"prefix":"10.1542","volume":"44","author":[{"given":"Christina","family":"Schutt","sequence":"first","affiliation":[{"name":"*Department of Pediatrics, Golisano Children\u2019s Hospital, University of Rochester, Rochester, NY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Siegel","sequence":"additional","affiliation":[{"name":"*Department of Pediatrics, Golisano Children\u2019s Hospital, University of Rochester, Rochester, NY"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2023,9,1]]},"reference":[{"issue":"8","key":"2023090103065545600_B1","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1038\/ni.3777","article-title":"The monogenic autoinflammatory diseases define new pathways in human innate immunity and inflammation","volume":"18","author":"Manthiram","year":"2017","journal-title":"Nat Immunol"},{"issue":"11","key":"2023090103065545600_B2","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1136\/annrheumdis-2017-212515","article-title":"Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study","volume":"77","author":"Ben-Chetrit","year":"2018","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"2023090103065545600_B3","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1038\/ng0997-25","article-title":"A candidate gene for familial Mediterranean fever","volume":"17","author":"Bernot","year":"1997","journal-title":"Nat Genet"},{"key":"2023090103065545600_B4","doi-asserted-by":"crossref","first-page":"1745","DOI":"10.3389\/fimmu.2019.01745","article-title":"The pyrin inflammasome in health and disease","volume":"10","author":"Schnappauf","year":"2019","journal-title":"Front Immunol"},{"issue":"3","key":"2023090103065545600_B5","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/j.chom.2016.07.018","article-title":"The Yersinia virulence factor YopM hijacks host kinases to inhibit type III effector-triggered activation of the pyrin inflammasome","volume":"20","author":"Chung","year":"2016","journal-title":"Cell Host Microbe"},{"issue":"10","key":"2023090103065545600_B6","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1002\/art.24458","article-title":"Familial Mediterranean fever in the world","volume":"61","author":"Ben-Chetrit","year":"2009","journal-title":"Arthritis Rheum"},{"key":"2023090103065545600_B7","unstructured":"Infevers: an online database for autoinflammatory mutations. 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