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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

Hinze CH, Foell D, Kessel C
Nat Rev Rheumatol 2023 Dec;19(12):778-789. Epub 2023 Nov 3 doi: 10.1038/s41584-023-01042-z. PMID: 37923864
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

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
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

Hinze CH, Foell D, Kessel C
Nat Rev Rheumatol 2023 Dec;19(12):778-789. Epub 2023 Nov 3 doi: 10.1038/s41584-023-01042-z. PMID: 37923864
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
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

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
Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Ravelli A, Consolaro A; Epidemiology, treatment and Outcome of Childhood Arthritis (EPOCA) Project, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Rheumatol Int 2018 Apr;38(Suppl 1):1-3. doi: 10.1007/s00296-018-3999-z. PMID: 29637373
Gmuca S, Weiss PF
Curr Opin Rheumatol 2015 Jul;27(4):364-72. doi: 10.1097/BOR.0000000000000185. PMID: 26002028Free PMC Article
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
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
Ravelli A, Consolaro A; Epidemiology, treatment and Outcome of Childhood Arthritis (EPOCA) Project, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Rheumatol Int 2018 Apr;38(Suppl 1):1-3. doi: 10.1007/s00296-018-3999-z. PMID: 29637373
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

Paik JJ, Lubin G, Gromatzky A, Mudd PN Jr, Ponda MP, Christopher-Stine L
Clin Exp Rheumatol 2023 Mar;41(2):348-358. Epub 2022 Jun 28 doi: 10.55563/clinexprheumatol/hxin6o. PMID: 35766013Free PMC Article
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
Goodman SM, Springer BD, Chen AF, Davis M, Fernandez DR, Figgie M, Finlayson H, George MD, Giles JT, Gilliland J, Klatt B, MacKenzie R, Michaud K, Miller A, Russell L, Sah A, Abdel MP, Johnson B, Mandl LA, Sculco P, Turgunbaev M, Turner AS, Yates A Jr, Singh JA
Arthritis Care Res (Hoboken) 2022 Sep;74(9):1399-1408. Epub 2022 Jun 19 doi: 10.1002/acr.24893. PMID: 35718887
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
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

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