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Juvenile onset Still disease

MedGen UID:
88662
Concept ID:
C0087031
Disease or Syndrome
Synonyms: Arthritis, Juvenile Systemic; Juvenile Onset Still Disease; Juvenile Onset Stills Disease; Juvenile Systemic Arthritis; Juvenile-Onset Still Disease; Juvenile-Onset Still's Disease; Juvenile-Onset Stills Disease; Still Disease, Juvenile Onset; Still Disease, Juvenile-Onset; Still's Disease, Juvenile Onset; Still's Disease, Juvenile-Onset; Stills Disease, Juvenile-Onset; Systemic Arthritis, Juvenile
SNOMED CT: Juvenile onset Still's disease (201796004)

Definition

An inflammatory disorder most often affecting children. It is characterized by the presence of arthritis, salmon-colored rash, spiking fevers, fatigue, and sore throats. [from NCI]

Term Hierarchy

Professional guidelines

PubMed

Aletaha D, Kerschbaumer A, Kastrati K, Dejaco C, Dougados M, McInnes IB, Sattar N, Stamm TA, Takeuchi T, Trauner M, van der Heijde D, Voshaar M, Winthrop KL, Ravelli A, Betteridge N, Burmester GR, Bijlsma JW, Bykerk V, Caporali R, Choy EH, Codreanu C, Combe B, Crow MK, de Wit M, Emery P, Fleischmann RM, Gabay C, Hetland ML, Hyrich KL, Iagnocco A, Isaacs JD, Kremer JM, Mariette X, Merkel PA, Mysler EF, Nash P, Nurmohamed MT, Pavelka K, Poor G, Rubbert-Roth A, Schulze-Koops H, Strangfeld A, Tanaka Y, Smolen JS
Ann Rheum Dis 2023 Jun;82(6):773-787. Epub 2022 Aug 11 doi: 10.1136/ard-2022-222784. PMID: 35953263
Onel K, Rumsey DG, Shenoi S
Rheum Dis Clin North Am 2021 Nov;47(4):545-563. Epub 2021 Sep 2 doi: 10.1016/j.rdc.2021.07.009. PMID: 34635291
Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P
Semin Arthritis Rheum 2021 Aug;51(4):858-874. Epub 2021 Jun 13 doi: 10.1016/j.semarthrit.2021.06.004. PMID: 34175791

Recent clinical studies

Etiology

Zaripova LN, Midgley A, Christmas SE, Beresford MW, Baildam EM, Oldershaw RA
Pediatr Rheumatol Online J 2021 Aug 23;19(1):135. doi: 10.1186/s12969-021-00629-8. PMID: 34425842Free PMC Article
Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P
Semin Arthritis Rheum 2021 Aug;51(4):858-874. Epub 2021 Jun 13 doi: 10.1016/j.semarthrit.2021.06.004. PMID: 34175791
Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Prakken B, Albani S, Martini A
Lancet 2011 Jun 18;377(9783):2138-49. doi: 10.1016/S0140-6736(11)60244-4. PMID: 21684384
Ravelli A, Martini A
Lancet 2007 Mar 3;369(9563):767-778. doi: 10.1016/S0140-6736(07)60363-8. PMID: 17336654

Diagnosis

Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F
Rheumatology (Oxford) 2019 Nov 1;58(Suppl 6):vi9-vi22. doi: 10.1093/rheumatology/kez350. PMID: 31769856Free PMC Article
Lee JJY, Schneider R
Pediatr Clin North Am 2018 Aug;65(4):691-709. doi: 10.1016/j.pcl.2018.04.005. PMID: 30031494
Prakken B, Albani S, Martini A
Lancet 2011 Jun 18;377(9783):2138-49. doi: 10.1016/S0140-6736(11)60244-4. PMID: 21684384
Ravelli A, Martini A
Lancet 2007 Mar 3;369(9563):767-778. doi: 10.1016/S0140-6736(07)60363-8. PMID: 17336654

Therapy

Gillard L, Pouchot J, Cohen-Aubart F, Koné-Paut I, Mouterde G, Michaud M, Reumaux H, Savey L, Belot A, Fautrel B, Mitrovic S
Rheumatology (Oxford) 2023 Apr 3;62(4):1594-1604. doi: 10.1093/rheumatology/keac440. PMID: 35920788
Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F
Rheumatology (Oxford) 2019 Nov 1;58(Suppl 6):vi9-vi22. doi: 10.1093/rheumatology/kez350. PMID: 31769856Free PMC Article
Lee JJY, Schneider R
Pediatr Clin North Am 2018 Aug;65(4):691-709. doi: 10.1016/j.pcl.2018.04.005. PMID: 30031494
Ravelli A, Martini A
Lancet 2007 Mar 3;369(9563):767-778. doi: 10.1016/S0140-6736(07)60363-8. PMID: 17336654

Prognosis

Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F
Rheumatology (Oxford) 2019 Nov 1;58(Suppl 6):vi9-vi22. doi: 10.1093/rheumatology/kez350. PMID: 31769856Free PMC Article
Saper VE, Chen G, Deutsch GH, Guillerman RP, Birgmeier J, Jagadeesh K, Canna S, Schulert G, Deterding R, Xu J, Leung AN, Bouzoubaa L, Abulaban K, Baszis K, Behrens EM, Birmingham J, Casey A, Cidon M, Cron RQ, De A, De Benedetti F, Ferguson I, Fishman MP, Goodman SI, Graham TB, Grom AA, Haines K, Hazen M, Henderson LA, Ho A, Ibarra M, Inman CJ, Jerath R, Khawaja K, Kingsbury DJ, Klein-Gitelman M, Lai K, Lapidus S, Lin C, Lin J, Liptzin DR, Milojevic D, Mombourquette J, Onel K, Ozen S, Perez M, Phillippi K, Prahalad S, Radhakrishna S, Reinhardt A, Riskalla M, Rosenwasser N, Roth J, Schneider R, Schonenberg-Meinema D, Shenoi S, Smith JA, Sönmez HE, Stoll ML, Towe C, Vargas SO, Vehe RK, Young LR, Yang J, Desai T, Balise R, Lu Y, Tian L, Bejerano G, Davis MM, Khatri P, Mellins ED; Childhood Arthritis and Rheumatology Research Alliance Registry Investigators
Ann Rheum Dis 2019 Dec;78(12):1722-1731. Epub 2019 Sep 27 doi: 10.1136/annrheumdis-2019-216040. PMID: 31562126Free PMC Article
Crayne CB, Beukelman T
Pediatr Clin North Am 2018 Aug;65(4):657-674. doi: 10.1016/j.pcl.2018.03.005. PMID: 30031492
Ravelli A, Martini A
Lancet 2007 Mar 3;369(9563):767-778. doi: 10.1016/S0140-6736(07)60363-8. PMID: 17336654

Clinical prediction guides

De Benedetti F, Grom AA, Brogan PA, Bracaglia C, Pardeo M, Marucci G, Eleftheriou D, Papadopoulou C, Schulert GS, Quartier P, Antón J, Laveille C, Frederiksen R, Asnaghi V, Ballabio M, Jacqmin P, de Min C
Ann Rheum Dis 2023 Jun;82(6):857-865. Epub 2023 Mar 31 doi: 10.1136/ard-2022-223739. PMID: 37001971Free PMC Article
Ruscitti P, Natoli V, Consolaro A, Caorsi R, Rosina S, Giancane G, Naddei R, Di Cola I, Di Muzio C, Berardicurti O, Iacono D, Pantano I, Rozza G, Rossi S, De Stefano L, Balduzzi S, Vitale A, Caso F, Costa L, Prete M, Navarini L, Iagnocco A, Atzeni F, Guggino G, Perosa F, Cantarini L, Frediani B, Montecucco C, Ciccia F, Cipriani P, Gattorno M, Giacomelli R, Ravelli A
Rheumatology (Oxford) 2022 Oct 6;61(10):4124-4129. doi: 10.1093/rheumatology/keac027. PMID: 35078234Free PMC Article
Efthimiou P, Kontzias A, Hur P, Rodha K, Ramakrishna GS, Nakasato P
Semin Arthritis Rheum 2021 Aug;51(4):858-874. Epub 2021 Jun 13 doi: 10.1016/j.semarthrit.2021.06.004. PMID: 34175791
Angeles-Han ST, Ringold S, Beukelman T, Lovell D, Cuello CA, Becker ML, Colbert RA, Feldman BM, Holland GN, Ferguson PJ, Gewanter H, Guzman J, Horonjeff J, Nigrovic PA, Ombrello MJ, Passo MH, Stoll ML, Rabinovich CE, Sen HN, Schneider R, Halyabar O, Hays K, Shah AA, Sullivan N, Szymanski AM, Turgunbaev M, Turner A, Reston J
Arthritis Care Res (Hoboken) 2019 Jun;71(6):703-716. Epub 2019 Apr 25 doi: 10.1002/acr.23871. PMID: 31021540Free PMC Article
Gmuca S, Weiss PF
Curr Opin Rheumatol 2015 Jul;27(4):364-72. doi: 10.1097/BOR.0000000000000185. PMID: 26002028Free PMC Article

Recent systematic reviews

Zeng L, Yang T, Yang K, Yu G, Li J, Xiang W, Chen H
Front Immunol 2022;13:891822. Epub 2022 Jul 22 doi: 10.3389/fimmu.2022.891822. PMID: 35935936Free PMC Article
Onel KB, Horton DB, Lovell DJ, Shenoi S, Cuello CA, Angeles-Han ST, Becker ML, Cron RQ, Feldman BM, Ferguson PJ, Gewanter H, Guzman J, Kimura Y, Lee T, Murphy K, Nigrovic PA, Ombrello MJ, Rabinovich CE, Tesher M, Twilt M, Klein-Gitelman M, Barbar-Smiley F, Cooper AM, Edelheit B, Gillispie-Taylor M, Hays K, Mannion ML, Peterson R, Flanagan E, Saad N, Sullivan N, Szymanski AM, Trachtman R, Turgunbaev M, Veiga K, Turner AS, Reston JT
Arthritis Rheumatol 2022 Apr;74(4):553-569. Epub 2022 Mar 1 doi: 10.1002/art.42037. PMID: 35233993Free PMC Article
McDonnell L, Gilkes A, Ashworth M, Rowland V, Harries TH, Armstrong D, White P
Gut Microbes 2021 Jan-Dec;13(1):1-18. doi: 10.1080/19490976.2020.1870402. PMID: 33651651Free PMC Article
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Kuntze G, Nesbitt C, Whittaker JL, Nettel-Aguirre A, Toomey C, Esau S, Doyle-Baker PK, Shank J, Brooks J, Benseler S, Emery CA
Arch Phys Med Rehabil 2018 Jan;99(1):178-193.e1. Epub 2017 Jul 18 doi: 10.1016/j.apmr.2017.05.030. PMID: 28729171

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