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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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVJuvenile onset Still disease

Professional guidelines

PubMed

Treatment of systemic juvenile idiopathic arthritis.
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
Juvenile Idiopathic Arthritis Treatment Updates.
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
Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies.
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

Uveitis Associated with Juvenile Idiopathic Arthritis.
van Meerwijk C, Kuiper J, van Straalen J, Ayuso VK, Wennink R, Haasnoot AM, Kouwenberg C, de Boer J
Ocul Immunol Inflamm 2023 Dec;31(10):1906-1914. Epub 2023 Dec 14 doi: 10.1080/09273948.2023.2278060. PMID: 37966463
Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches.
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
Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies.
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
Autoinflammatory syndromes.
Cush JJ
Dermatol Clin 2013 Jul;31(3):471-80. doi: 10.1016/j.det.2013.05.001. PMID: 23827249Free PMC Article
Juvenile idiopathic arthritis.
Prakken B, Albani S, Martini A
Lancet 2011 Jun 18;377(9783):2138-49. doi: 10.1016/S0140-6736(11)60244-4. PMID: 21684384

Diagnosis

EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.
Fautrel B, Mitrovic S, De Matteis A, Bindoli S, Antón J, Belot A, Bracaglia C, Constantin T, Dagna L, Di Bartolo A, Feist E, Foell D, Gattorno M, Georgin-Lavialle S, Giacomelli R, Grom AA, Jamilloux Y, Laskari K, Lazar C, Minoia F, Nigrovic PA, Oliveira Ramos F, Ozen S, Quartier P, Ruscitti P, Sag E, Savic S, Truchetet ME, Vastert SJ, Wilhelmer TC, Wouters C, Carmona L, De Benedetti F
Ann Rheum Dis 2024 Nov 14;83(12):1614-1627. doi: 10.1136/ard-2024-225851. PMID: 39317417Free PMC Article
Uveitis Associated with Juvenile Idiopathic Arthritis.
van Meerwijk C, Kuiper J, van Straalen J, Ayuso VK, Wennink R, Haasnoot AM, Kouwenberg C, de Boer J
Ocul Immunol Inflamm 2023 Dec;31(10):1906-1914. Epub 2023 Dec 14 doi: 10.1080/09273948.2023.2278060. PMID: 37966463
Updates in Juvenile Idiopathic Arthritis.
McCurdy D, Parsa MF
Adv Pediatr 2021 Aug;68:143-170. doi: 10.1016/j.yapd.2021.05.014. PMID: 34243850
Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies.
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
Juvenile idiopathic arthritis.
Prakken B, Albani S, Martini A
Lancet 2011 Jun 18;377(9783):2138-49. doi: 10.1016/S0140-6736(11)60244-4. PMID: 21684384

Therapy

Recent advances and evolving concepts in Still's disease.
Ruscitti P, Cantarini L, Nigrovic PA, McGonagle D, Giacomelli R
Nat Rev Rheumatol 2024 Feb;20(2):116-132. Epub 2024 Jan 11 doi: 10.1038/s41584-023-01065-6. PMID: 38212542
Treatment of systemic juvenile idiopathic arthritis.
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
Updates in Juvenile Idiopathic Arthritis.
McCurdy D, Parsa MF
Adv Pediatr 2021 Aug;68:143-170. doi: 10.1016/j.yapd.2021.05.014. PMID: 34243850
Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies.
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
Autoinflammatory syndromes.
Cush JJ
Dermatol Clin 2013 Jul;31(3):471-80. doi: 10.1016/j.det.2013.05.001. PMID: 23827249Free PMC Article

Prognosis

Efficacy and safety of emapalumab in macrophage activation syndrome.
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
Juvenile idiopathic arthritis-associated uveitis.
Sen ES, Ramanan AV
Clin Immunol 2020 Feb;211:108322. Epub 2019 Dec 9 doi: 10.1016/j.clim.2019.108322. PMID: 31830532
Juvenile idiopathic arthritis.
Ravelli A, Martini A
Lancet 2007 Mar 3;369(9563):767-778. doi: 10.1016/S0140-6736(07)60363-8. PMID: 17336654
Juvenile chronic arthritis.
Woo P, Wedderburn LR
Lancet 1998 Mar 28;351(9107):969-73. PMID: 9734957
Still's disease.
Br Med J 1968 Oct 12;4(5623):65-6. PMID: 5696544Free PMC Article

Clinical prediction guides

Efficacy and safety of emapalumab in macrophage activation syndrome.
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
Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies.
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
2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis.
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
Preface.
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
Juvenile spondyloarthritis.
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

The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).
Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW; HLH/MAS task force
Ann Rheum Dis 2023 Oct;82(10):1271-1285. Epub 2023 Jul 24 doi: 10.1136/ard-2023-224123. PMID: 37487610Free PMC Article
Efficacy and Safety of Curcumin and Curcuma longa Extract in the Treatment of Arthritis: A Systematic Review and Meta-Analysis of Randomized Controlled Trial.
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
2022 American College of Rheumatology/American Association of Hip and Knee Surgeons Guideline for the Perioperative Management of Antirheumatic Medication in Patients With Rheumatic Diseases Undergoing Elective Total Hip or Total Knee Arthroplasty.
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
2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis.
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 Care Res (Hoboken) 2022 Apr;74(4):521-537. Epub 2022 Mar 1 doi: 10.1002/acr.24853. PMID: 35233986Free PMC Article
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
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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