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Periodic fever syndrome

MedGen UID:
855463
Concept ID:
C3889979
Disease or Syndrome
Synonyms: Periodic Fever Syndrome; periodic fever syndrome
 
Monarch Initiative: MONDO:0015137
Orphanet: ORPHA101995

Definition

Fevers of unknown etiology recurring over months or years. [from NCI]

Professional guidelines

PubMed

Malcova H, Strizova Z, Milota T, Striz I, Sediva A, Cebecauerova D, Horvath R
Front Immunol 2020;11:619257. Epub 2021 Feb 1 doi: 10.3389/fimmu.2020.619257. PMID: 33603750Free PMC Article
Amarilyo G, Rothman D, Manthiram K, Edwards KM, Li SC, Marshall GS, Yildirim-Toruner C, Haines K, Ferguson PJ, Lionetti G, Cherian J, Zhao Y, DeLaMora P, Syverson G, Nativ S, Twilt M, Michelow IC, Stepanovskiy Y, Thatayatikom A, Harel L, Akoghlanian S, Tucker L, Marques MC, Srinivasalu H, Propst EJ, Licameli GR, Dedeoglu F, Lapidus S; CARRA PFAPA Consensus Treatment Plan Workgroup
Pediatr Rheumatol Online J 2020 Apr 15;18(1):31. doi: 10.1186/s12969-020-00424-x. PMID: 32293478Free PMC Article
Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N; Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2019 Aug;78(8):1025-1032. Epub 2019 Apr 24 doi: 10.1136/annrheumdis-2019-215048. PMID: 31018962

Recent clinical studies

Etiology

Schutt C, Siegel DM
Pediatr Rev 2023 Sep 1;44(9):481-490. doi: 10.1542/pir.2022-005635. PMID: 37653132
Amarilyo G, Rothman D, Manthiram K, Edwards KM, Li SC, Marshall GS, Yildirim-Toruner C, Haines K, Ferguson PJ, Lionetti G, Cherian J, Zhao Y, DeLaMora P, Syverson G, Nativ S, Twilt M, Michelow IC, Stepanovskiy Y, Thatayatikom A, Harel L, Akoghlanian S, Tucker L, Marques MC, Srinivasalu H, Propst EJ, Licameli GR, Dedeoglu F, Lapidus S; CARRA PFAPA Consensus Treatment Plan Workgroup
Pediatr Rheumatol Online J 2020 Apr 15;18(1):31. doi: 10.1186/s12969-020-00424-x. PMID: 32293478Free PMC Article
Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N; Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2019 Aug;78(8):1025-1032. Epub 2019 Apr 24 doi: 10.1136/annrheumdis-2019-215048. PMID: 31018962
Ozen S
Pediatr Nephrol 2019 Dec;34(12):2449-2456. Epub 2018 Dec 14 doi: 10.1007/s00467-018-4155-4. PMID: 30552566
Grateau G
Acta Clin Belg 2006 Sep-Oct;61(5):264-9. doi: 10.1179/acb.2006.045. PMID: 17240743

Diagnosis

Schutt C, Siegel DM
Pediatr Rev 2023 Sep 1;44(9):481-490. doi: 10.1542/pir.2022-005635. PMID: 37653132
Sánchez-Bernal J, Conejero C, Conejero R
Actas Dermosifiliogr (Engl Ed) 2020 Jul-Aug;111(6):471-480. Epub 2020 May 22 doi: 10.1016/j.ad.2019.09.004. PMID: 32451064
Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N; Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2019 Aug;78(8):1025-1032. Epub 2019 Apr 24 doi: 10.1136/annrheumdis-2019-215048. PMID: 31018962
Ozen S
Pediatr Nephrol 2019 Dec;34(12):2449-2456. Epub 2018 Dec 14 doi: 10.1007/s00467-018-4155-4. PMID: 30552566
Grateau G
Acta Clin Belg 2006 Sep-Oct;61(5):264-9. doi: 10.1179/acb.2006.045. PMID: 17240743

Therapy

Lantto U, Tapiainen T, Pokka T, Koivunen P, Helminen M, Piitulainen J, Rekola J, Uhari M, Renko M
Laryngoscope 2024 Feb;134(2):968-972. Epub 2023 Jul 21 doi: 10.1002/lary.30863. PMID: 37477273
Soriano A, Soriano M, Espinosa G, Manna R, Emmi G, Cantarini L, Hernández-Rodríguez J
Front Immunol 2020;11:865. Epub 2020 Jun 3 doi: 10.3389/fimmu.2020.00865. PMID: 32655539Free PMC Article
Amarilyo G, Rothman D, Manthiram K, Edwards KM, Li SC, Marshall GS, Yildirim-Toruner C, Haines K, Ferguson PJ, Lionetti G, Cherian J, Zhao Y, DeLaMora P, Syverson G, Nativ S, Twilt M, Michelow IC, Stepanovskiy Y, Thatayatikom A, Harel L, Akoghlanian S, Tucker L, Marques MC, Srinivasalu H, Propst EJ, Licameli GR, Dedeoglu F, Lapidus S; CARRA PFAPA Consensus Treatment Plan Workgroup
Pediatr Rheumatol Online J 2020 Apr 15;18(1):31. doi: 10.1186/s12969-020-00424-x. PMID: 32293478Free PMC Article
Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N; Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2019 Aug;78(8):1025-1032. Epub 2019 Apr 24 doi: 10.1136/annrheumdis-2019-215048. PMID: 31018962
Hausmann JS, Dedeoglu F
Dermatol Clin 2013 Jul;31(3):481-94. Epub 2013 Jun 2 doi: 10.1016/j.det.2013.04.003. PMID: 23827250

Prognosis

Schutt C, Siegel DM
Pediatr Rev 2023 Sep 1;44(9):481-490. doi: 10.1542/pir.2022-005635. PMID: 37653132
Manthiram K
Curr Opin Rheumatol 2023 Nov 1;35(6):423-428. Epub 2023 Jul 17 doi: 10.1097/BOR.0000000000000956. PMID: 37467064Free PMC Article
Banday AZ, Joshi V, Arora K, Sadanand R, Basu S, Pilania RK, Jindal AK, Vignesh P, Gupta A, Sharma S, Dhaliwal M, Rawat A, Singh S, Suri D
Front Immunol 2022;13:958473. Epub 2022 Sep 20 doi: 10.3389/fimmu.2022.958473. PMID: 36203600Free PMC Article
Alghamdi M
Curr Rheumatol Rep 2018 Nov 17;20(12):87. doi: 10.1007/s11926-018-0788-3. PMID: 30446874
van der Hilst JC, Simon A, Drenth JP
Clin Exp Med 2005 Oct;5(3):87-98. doi: 10.1007/s10238-005-0071-6. PMID: 16284730

Clinical prediction guides

Lantto U, Tapiainen T, Pokka T, Koivunen P, Helminen M, Piitulainen J, Rekola J, Uhari M, Renko M
Laryngoscope 2024 Feb;134(2):968-972. Epub 2023 Jul 21 doi: 10.1002/lary.30863. PMID: 37477273
Manthiram K
Curr Opin Rheumatol 2023 Nov 1;35(6):423-428. Epub 2023 Jul 17 doi: 10.1097/BOR.0000000000000956. PMID: 37467064Free PMC Article
McNaughton P, Willcocks S, Lum SH, Whitehead B, Peake J, Preece K
J Paediatr Child Health 2022 Mar;58(3):404-408. Epub 2021 Sep 9 doi: 10.1111/jpc.15722. PMID: 34499401
Ter Haar NM, Annink KV, Al-Mayouf SM, Amaryan G, Anton J, Barron KS, Benseler SM, Brogan PA, Cantarini L, Cattalini M, Cochino AV, De Benedetti F, Dedeoglu F, De Jesus AA, Della Casa Alberighi O, Demirkaya E, Dolezalova P, Durrant KL, Fabio G, Gallizzi R, Goldbach-Mansky R, Hachulla E, Hentgen V, Herlin T, Hofer M, Hoffman HM, Insalaco A, Jansson AF, Kallinich T, Koné-Paut I, Kozlova A, Kuemmerle-Deschner JB, Lachmann HJ, Laxer RM, Martini A, Nielsen S, Nikishina I, Ombrello AK, Ozen S, Papadopoulou-Alataki E, Quartier P, Rigante D, Russo R, Simon A, Trachana M, Uziel Y, Ravelli A, Gattorno M, Frenkel J
Ann Rheum Dis 2017 May;76(5):821-830. Epub 2016 Nov 3 doi: 10.1136/annrheumdis-2016-210092. PMID: 27811147
Stoffels M, Simon A
Curr Opin Rheumatol 2011 Sep;23(5):419-23. doi: 10.1097/BOR.0b013e328349c3b1. PMID: 21760510

Recent systematic reviews

Silva GD, Mahler JV, da Silva Junior SRP, Mendonça LO, de Sá Barreto Lima PLG, Nóbrega PR, Kok F, Freua F
BMC Neurol 2024 Apr 17;24(1):130. doi: 10.1186/s12883-024-03621-3. PMID: 38632524Free PMC Article
Yao Q, Englund KA, Hayden SP, Tomecki KJ
Semin Arthritis Rheum 2012 Feb;41(4):611-7. Epub 2011 Sep 25 doi: 10.1016/j.semarthrit.2011.07.008. PMID: 21943788

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