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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]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeriodic fever syndrome

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
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
Manthiram K, Preite S, Dedeoglu F, Demir S, Ozen S, Edwards KM, Lapidus S, Katz AE; Genomic Ascertainment Cohort, Feder HM Jr, Lawton M, Licameli GR, Wright PF, Le J, Barron KS, Ombrello AK, Barham B, Romeo T, Jones A, Srinivasalu H, Mudd PA, DeBiasi RL, Gül A, Marshall GS, Jones OY, Chandrasekharappa SC, Stepanovskiy Y, Ferguson PJ, Schwartzberg PL, Remmers EF, Kastner DL
Proc Natl Acad Sci U S A 2020 Jun 23;117(25):14405-14411. Epub 2020 Jun 9 doi: 10.1073/pnas.2002051117. PMID: 32518111Free 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

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
Soon GS, Laxer RM
Can Fam Physician 2017 Oct;63(10):756-762. PMID: 29025800Free PMC Article
Sag E, Bilginer Y, Ozen S
Curr Rheumatol Rep 2017 Jul;19(7):41. doi: 10.1007/s11926-017-0670-8. PMID: 28631068

Therapy

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
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
Thomas KT, Edwards KM
Pediatr Infect Dis J 1999 Jan;18(1):68-9. doi: 10.1097/00006454-199901000-00017. PMID: 9951985

Prognosis

Schutt C, Siegel DM
Pediatr Rev 2023 Sep 1;44(9):481-490. doi: 10.1542/pir.2022-005635. PMID: 37653132
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
Graňák K, Jeseňák M, Vnučák M, Skálová P, Laca Ľ, Mokáň M, Dedinská I
Clin Rheumatol 2021 Feb;40(2):763-768. Epub 2020 Jul 11 doi: 10.1007/s10067-020-05283-1. PMID: 32654081
Alghamdi M
Curr Rheumatol Rep 2018 Nov 17;20(12):87. doi: 10.1007/s11926-018-0788-3. PMID: 30446874
Soon GS, Laxer RM
Can Fam Physician 2017 Oct;63(10):756-762. PMID: 29025800Free PMC Article

Clinical prediction guides

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
Zhao M, Luo Y, Wu D, Yang Y, Sun Y, Wang R, Shen M
Rheumatology (Oxford) 2020 Aug 1;59(8):1969-1974. doi: 10.1093/rheumatology/kez569. PMID: 31774538
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, Jongekrijg J, Remijn T, Kok N, van der Meer JW, Simon A
Rheumatology (Oxford) 2015 Feb;54(2):363-8. Epub 2014 Aug 29 doi: 10.1093/rheumatology/keu341. PMID: 25173351
Feder HM Jr
Curr Opin Pediatr 2000 Jun;12(3):253-6. doi: 10.1097/00008480-200006000-00014. PMID: 10836162

Recent systematic reviews

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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