{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T19:07:47Z","timestamp":1791400067293,"version":"4.3.3"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2016,6,1]],"date-time":"2016-06-01T00:00:00Z","timestamp":1464739200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2017,2]]},"DOI":"10.1007\/s10067-016-3317-7","type":"journal-article","created":{"date-parts":[[2016,6,1]],"date-time":"2016-06-01T04:14:31Z","timestamp":1464754471000},"page":"463-468","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":76,"title":["A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome"],"prefix":"10.1007","volume":"36","author":[{"given":"Donato","family":"Rigante","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Vitale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco Francesco","family":"Natale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Lopalco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Andreozzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Frediani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"D\u2019Errico","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florenzo","family":"Iannone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Cantarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2016,6,1]]},"reference":[{"key":"3317_CR1","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1111\/j.1651-2227.2009.01554.x","volume":"99","author":"HM Feder","year":"2010","unstructured":"Feder HM, Salazar JC (2010) A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr 99:178\u201384","journal-title":"Acta Paediatr"},{"key":"3317_CR2","first-page":"1","volume":"14","author":"D Rigante","year":"2010","unstructured":"Rigante D (2010) The protean visage of systemic autoinflammatory syndromes: a challenge for inter-professional collaboration. Eur Rev Med Pharmacol Sci 14:1\u201318","journal-title":"Eur Rev Med Pharmacol Sci"},{"key":"3317_CR3","doi-asserted-by":"crossref","first-page":"563876","DOI":"10.1155\/2015\/563876","volume":"2015","author":"B Kraszewska-G\u0142omba","year":"2015","unstructured":"Kraszewska-G\u0142omba B, Matkowska-Kocjan A, Szenborn L (2015) The pathogenesis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: a review of current research. Mediators Inflamm 2015:563876","journal-title":"Mediators Inflamm"},{"key":"3317_CR4","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1136\/adc.2005.084731","volume":"91","author":"D Tasher","year":"2006","unstructured":"Tasher D, Somekh E, Dalal I (2006) PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child 91:981\u20134","journal-title":"Arch Dis Child"},{"key":"3317_CR5","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1093\/rheumatology\/ket460","volume":"53","author":"M Hofer","year":"2014","unstructured":"Hofer M, Pillet P, Cochard MM, Berg S, Krol P, Kone-Paut I et al (2014) International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford) 53:1125\u20139","journal-title":"Rheumatology (Oxford)"},{"key":"3317_CR6","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0022-3476(87)80285-8","volume":"110","author":"GS Marshall","year":"1987","unstructured":"Marshall GS, Edwards KM, Butler J, Lawton AR (1987) Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 110:43\u20136","journal-title":"J Pediatr"},{"key":"3317_CR7","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/S0022-3476(99)70321-5","volume":"135","author":"KT Thomas","year":"1999","unstructured":"Thomas KT, Feder HM Jr, Lawton AR (1999) Periodic fever syndrome in children. J Pediatr 135:15\u201321","journal-title":"J Pediatr"},{"key":"3317_CR8","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1016\/j.jpeds.2011.06.004","volume":"159","author":"VM Wurster","year":"2011","unstructured":"Wurster VM, Carlucci JG, Feder HMJR, Edwards KM (2011) Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr 159:958\u201364","journal-title":"J Pediatr"},{"key":"3317_CR9","doi-asserted-by":"crossref","first-page":"570418","DOI":"10.1155\/2015\/570418","volume":"2015","author":"M Cattalini","year":"2015","unstructured":"Cattalini M, Soliani M, Rigante D, Lopalco G, Iannone F, Galeazzi M et al (2015) Basic characteristics of adults with periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome in comparison with the typical pediatric expression of disease. Mediators Inflamm 2015:570418","journal-title":"Mediators Inflamm"},{"key":"3317_CR10","first-page":"269","volume":"30","author":"L Cantarini","year":"2012","unstructured":"Cantarini L, Vitale A, Bartolomei B, Galeazzi M, Rigante D (2012) Diagnosis of PFAPA syndrome applied to a cohort of 17 adults with unexplained recurrent fevers. Clin Exp Rheumatol 30:269\u201371","journal-title":"Clin Exp Rheumatol"},{"key":"3317_CR11","first-page":"59","volume":"30","author":"L Cantarini","year":"2012","unstructured":"Cantarini L, Vitale A, Galeazzi M, Frediani B (2012) A case of resistant adult-onset periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome responsive to anakinra. Clin Exp Rheumatol 30:59","journal-title":"Clin Exp Rheumatol"},{"key":"3317_CR12","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.12722","author":"G Lopalco","year":"2015","unstructured":"Lopalco G, Rigante D, Vitale A, Caso F, Iannone F, Cantarini L (2015) Canakinumab efficacy in refractory adult-onset PFAPA syndrome. Int J Rheum Dis. doi: 10.1111\/1756-185X.12722","journal-title":"Int J Rheum Dis"},{"key":"3317_CR13","doi-asserted-by":"crossref","first-page":"223","DOI":"10.2169\/internalmedicine.50.4421","volume":"50","author":"M Colotto","year":"2011","unstructured":"Colotto M, Maranghi M, Durante C, Rossetti M, Renzi A, Anatra MG (2011) PFAPA syndrome in a young adult with a history of tonsillectomy. Intern Med 50:223\u20135","journal-title":"Intern Med"},{"key":"3317_CR14","first-page":"358","volume":"10","author":"S Padeh","year":"2008","unstructured":"Padeh S, Stoffman N, Berkun Y (2008) Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr Med Assoc J 10:358\u201360","journal-title":"Isr Med Assoc J"},{"key":"3317_CR15","doi-asserted-by":"crossref","first-page":"e721","DOI":"10.1542\/peds.2009-0088","volume":"124","author":"M Gattorno","year":"2009","unstructured":"Gattorno M, Caorsi R, Meini A, Cattalini M, Federici S, Zulian F et al (2009) Differentiating PFAPA syndrome from monogenic periodic fevers. Pediatrics 124:e721\u20138","journal-title":"Pediatrics"},{"key":"3317_CR16","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1097\/INF.0000000000000346","volume":"33","author":"S Esposito","year":"2014","unstructured":"Esposito S, Bianchini S, Fattizzo M, Baggi E, Marchisio P, Rigante D (2014) The enigma of periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Pediatr Infect Dis J 33:650\u20132","journal-title":"Pediatr Infect Dis J"},{"key":"3317_CR17","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1136\/annrheumdis-2011-201268","volume":"72","author":"N Haar ter","year":"2013","unstructured":"ter Haar N, Lachmann H, \u00d6zen S, Woo P, Uziel Y, Modesto C et al (2013) Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review. Ann Rheum Dis 72:678\u201385","journal-title":"Ann Rheum Dis"},{"key":"3317_CR18","first-page":"90","volume":"17","author":"S Stojanov","year":"2006","unstructured":"Stojanov S, Hoffmann F, K\u00e9ry A, Renner ED, Hartl D, Lohse P et al (2006) Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response. Eur Cytokine Netw 17:90\u20137","journal-title":"Eur Cytokine Netw"},{"key":"3317_CR19","doi-asserted-by":"crossref","first-page":"1635","DOI":"10.1016\/j.jaci.2012.07.043","volume":"131","author":"L Kolly","year":"2013","unstructured":"Kolly L, Busso N, von Scheven-Gete A, Bagnoud N, Moix I et al (2013) Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome is linked to dysregulated monocyte IL-1beta production. J Allergy Clin Immunol 131:1635\u201343","journal-title":"J Allergy Clin Immunol"},{"key":"3317_CR20","doi-asserted-by":"crossref","first-page":"7148","DOI":"10.1073\/pnas.1103681108","volume":"108","author":"S Stojanov","year":"2011","unstructured":"Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA et al (2011) Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci U S A 108:7148\u201353","journal-title":"Proc Natl Acad Sci U S A"},{"key":"3317_CR21","first-page":"RA179","volume":"15","author":"D Rigante","year":"2009","unstructured":"Rigante D (2009) Autoinflammatory syndromes behind the scenes of recurrent fevers in children. Med Sci Monit 15:RA179\u201387","journal-title":"Med Sci Monit"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-016-3317-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10067-016-3317-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-016-3317-7","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-016-3317-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,17]],"date-time":"2024-06-17T07:48:24Z","timestamp":1718610504000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10067-016-3317-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,6,1]]},"references-count":21,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2017,2]]}},"alternative-id":["3317"],"URL":"https:\/\/doi.org\/10.1007\/s10067-016-3317-7","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,6,1]]}}}