{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,9]],"date-time":"2026-11-09T16:35:04Z","timestamp":1794242104717,"version":"4.9.0"},"reference-count":24,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T00:00:00Z","timestamp":1766966400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T00:00:00Z","timestamp":1766966400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"DOI":"10.1186\/s12969-025-01157-5","type":"journal-article","created":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T16:28:27Z","timestamp":1767025707000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Symptom course of PFAPA syndrome"],"prefix":"10.1186","volume":"23","author":[{"given":"Mathilde","family":"Labouret","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00e8s","family":"Elhani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien","family":"Cavelot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glory","family":"Dingulu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurine","family":"Jouret","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ai Tien","family":"Nguyen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrien","family":"Subervie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Sophie","family":"Tavernier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Vinit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Hentgen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,12,29]]},"reference":[{"issue":"2","key":"1157_CR1","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1111\/apa.12069","volume":"102","author":"J F\u00f8rsvoll","year":"2013","unstructured":"F\u00f8rsvoll J, Kristoffersen EK, \u00d8ymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Paediatr f\u00e9vr. 2013;102(2):187\u201392.","journal-title":"Acta Paediatr f\u00e9vr"},{"issue":"1","key":"1157_CR2","doi-asserted-by":"publisher","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. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr Janv. 1987;110(1):43\u20136.","journal-title":"J Pediatr Janv"},{"issue":"1","key":"1157_CR3","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1016\/S0022-3476(99)70321-5","volume":"135","author":"KT Thomas","year":"1999","unstructured":"Thomas KT, Feder HM, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr Juill. 1999;135(1):15\u201321.","journal-title":"J Pediatr Juill"},{"issue":"8","key":"1157_CR4","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1136\/annrheumdis-2019-215048","volume":"78","author":"M Gattorno","year":"2019","unstructured":"Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, et al. Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis ao\u00fbt. 2019;78(8):1025\u201332.","journal-title":"Ann Rheum Dis ao\u00fbt"},{"issue":"6","key":"1157_CR5","doi-asserted-by":"publisher","first-page":"958","DOI":"10.1016\/j.jpeds.2011.06.004","volume":"159","author":"VM Wurster","year":"2011","unstructured":"Wurster VM, Carlucci JG, Feder HM, Edwards KM. Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr d\u00e9c. 2011;159(6):958\u201364.","journal-title":"J Pediatr d\u00e9c"},{"issue":"1","key":"1157_CR6","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1186\/s12969-016-0101-9","volume":"14","author":"F Vanoni","year":"2016","unstructured":"Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J 27 Juin. 2016;14(1):38.","journal-title":"Pediatr Rheumatol Online J 27 Juin"},{"issue":"4","key":"1157_CR7","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1179\/2046905515Y.0000000051","volume":"36","author":"F Erdogan","year":"2016","unstructured":"Erdogan F, Kulak K, \u00d6zt\u00fcrk O, \u0130pek \u0130\u00d6, Ceran \u00d6, Seven H. Surgery vs medical treatment in the management of PFAPA syndrome: a comparative trial. Paediatr Int Child Health Nov. 2016;36(4):270\u20134.","journal-title":"Paediatr Int Child Health Nov"},{"key":"1157_CR8","doi-asserted-by":"crossref","unstructured":"Grimwood C, Kone-Paut I, Piram M, Rossi-Semerano L, Hentgen V. Health-related quality of life in children with PFAPA syndrome. Orphanet J Rare Dis. 9 ao\u00fbt. 2018;13(1):132.","DOI":"10.1186\/s13023-018-0878-3"},{"issue":"5","key":"1157_CR9","doi-asserted-by":"publisher","first-page":"678","DOI":"10.1136\/annrheumdis-2011-201268","volume":"72","author":"N Ter Haar","year":"2013","unstructured":"Ter Haar N, Lachmann H, \u00d6zen S, Woo P, Uziel Y, Modesto C, et al. Treatment of autoinflammatory diseases: results from the Eurofever registry and a literature review. Ann Rheum Dis Mai. 2013;72(5):678\u201385.","journal-title":"Ann Rheum Dis Mai"},{"issue":"6","key":"1157_CR10","doi-asserted-by":"publisher","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. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatol (Oxford) Juin. 2014;53(6):1125\u20139.","journal-title":"Rheumatol (Oxford) Juin"},{"key":"1157_CR11","doi-asserted-by":"crossref","unstructured":"Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev. 30 d\u00e9c. 2019;12(12):CD008669.","DOI":"10.1002\/14651858.CD008669.pub3"},{"issue":"7","key":"1157_CR12","first-page":"E2149","volume":"131","author":"U Lantto","year":"2021","unstructured":"Lantto U, Koivunen P, Tapiainen T, Renko M, Periodic, Fever. Aphthous Stomatitis, Pharyngitis, and cervical adenitis syndrome: relapse and tonsillar regrowth after childhood tonsillectomy. Laryngoscope Juill. 2021;131(7):E2149\u201352.","journal-title":"Laryngoscope Juill"},{"issue":"10","key":"1157_CR13","doi-asserted-by":"publisher","first-page":"902","DOI":"10.1001\/2013.jamaoto.313","volume":"138","author":"G Licameli","year":"2012","unstructured":"Licameli G, Lawton M, Kenna M, Dedeoglu F. Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome. Arch Otolaryngol Head Neck Surg Oct. 2012;138(10):902\u20136.","journal-title":"Arch Otolaryngol Head Neck Surg Oct"},{"issue":"3","key":"1157_CR14","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/S2213-8587(15)00380-0","volume":"4","author":"AC Latronico","year":"2016","unstructured":"Latronico AC, Brito VN, Carel JC. Causes, diagnosis, and treatment of central precocious puberty. Lancet Diabetes Endocrinol Mars. 2016;4(3):265\u201374.","journal-title":"Lancet Diabetes Endocrinol Mars"},{"issue":"6","key":"1157_CR15","doi-asserted-by":"publisher","first-page":"1739","DOI":"10.1210\/clinem\/dgac054","volume":"107","author":"J Harrington","year":"2022","unstructured":"Harrington J, Palmert MR. An approach to the patient with delayed puberty. J Clin Endocrinol Metab 17 Mai. 2022;107(6):1739\u201350.","journal-title":"J Clin Endocrinol Metab 17 Mai"},{"issue":"235","key":"1157_CR16","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1136\/adc.44.235.291","volume":"44","author":"WA Marshall","year":"1969","unstructured":"Marshall WA, Tanner JM. Variations in pattern of pubertal changes in girls. Arch Dis Child Juin. 1969;44(235):291\u2013303.","journal-title":"Arch Dis Child Juin"},{"issue":"239","key":"1157_CR17","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1136\/adc.45.239.13","volume":"45","author":"WA Marshall","year":"1970","unstructured":"Marshall WA, Tanner JM. Variations in the pattern of pubertal changes in boys. Arch Dis Child f\u00e9vr. 1970;45(239):13\u201323.","journal-title":"Arch Dis Child f\u00e9vr"},{"issue":"1","key":"1157_CR18","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1016\/S0022-3476(99)70335-5","volume":"135","author":"S Padeh","year":"1999","unstructured":"Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr Juill. 1999;135(1):98\u2013101.","journal-title":"J Pediatr Juill"},{"key":"1157_CR19","doi-asserted-by":"crossref","unstructured":"Sparud-Lundin C, Berg S, Fasth A, Karlsson A, Wekell P. From uncertainty to gradually managing and awaiting recovery of a periodic condition- a qualitative study of parents\u00b4 experiences of PFAPA syndrome. BMC pediatr [Internet]. 8 Avr 2019 [cit\u00e9 4 f\u00e9vr 2024];19:99. Disponible sur: https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC6454720\/.","DOI":"10.1186\/s12887-019-1458-y"},{"issue":"1","key":"1157_CR20","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1111\/all.14477","volume":"76","author":"M Hara","year":"2021","unstructured":"Hara M, Morimoto N, Suzuki N, Tsuchihashi N, Komori M, Yoshihama K, et al. Transcriptome analysis reveals two distinct endotypes and putative immune pathways in tonsils from children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. Allergy Janv. 2021;76(1):359\u201363.","journal-title":"Allergy Janv"},{"issue":"5","key":"1157_CR21","doi-asserted-by":"publisher","first-page":"1309","DOI":"10.1007\/s10067-017-3773-8","volume":"37","author":"K Manthiram","year":"2018","unstructured":"Manthiram K, Correa H, Boyd K, Roland J, Edwards K. Unique histologic features of tonsils from patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Clin Rheumatol Mai. 2018;37(5):1309\u201317.","journal-title":"Clin Rheumatol Mai"},{"issue":"6","key":"1157_CR22","doi-asserted-by":"publisher","first-page":"523","DOI":"10.1111\/apm.12383","volume":"123","author":"U Lantto","year":"2015","unstructured":"Lantto U, Koivunen P, Tapiainen T, Glumoff V, Hirvikoski P, Uhari M, et al. Microbes of the tonsils in PFAPA (Periodic Fever, aphtous stomatitis, pharyngitis and Adenitis) syndrome - a possible trigger of febrile episodes. APMIS Juin. 2015;123(6):523\u20139.","journal-title":"APMIS Juin"},{"issue":"1","key":"1157_CR23","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1007\/s00296-022-05210-4","volume":"43","author":"ED Gozen","year":"2023","unstructured":"Gozen ED, Yildiz M, Kara S, Tevetoglu F, Haslak F, Adrovic A, et al. Long-term efficacy of tonsillectomy\/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of Familial mediterranean fever. Rheumatol Int Janv. 2023;43(1):137\u201345.","journal-title":"Rheumatol Int Janv"},{"issue":"3","key":"1157_CR24","doi-asserted-by":"publisher","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. PFAPA syndrome in a young adult with a history of tonsillectomy. Intern Med. 2011;50(3):223\u20135.","journal-title":"Intern Med"}],"container-title":["Pediatric Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-025-01157-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12969-025-01157-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12969-025-01157-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,29]],"date-time":"2025-12-29T16:28:28Z","timestamp":1767025708000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s12969-025-01157-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,29]]},"references-count":24,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["1157"],"URL":"https:\/\/doi.org\/10.1186\/s12969-025-01157-5","relation":{},"ISSN":["1546-0096"],"issn-type":[{"value":"1546-0096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,29]]},"assertion":[{"value":"21 April 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 September 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 December 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The JIR cohort protocol was approved by the French Ethics Committee (CCTIRS) on April 21, 2015 (decision number 14.302). The electronic form of the JIR cohort was approved by the National Commission of data processing and liberties (CNIL) on March 27, 2015 (decision number DR-2015-218). All patients or their guardian were not opposed for inclusion and the storage of their medical data in the JIR cohort. They were informed that collected data might be used for research studies in compliance with privacy rules.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"126"}}