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Juvenile idiopathic arthritis(JUVAR)

MedGen UID:
760659
•Concept ID:
C3495559
•
Disease or Syndrome
Synonyms: Arthritides, Childhood; Arthritis, Childhood; Arthritis, Juvenile; Arthritis, Juvenile Chronic; Arthritis, Juvenile Idiopathic; Arthritis, Juvenile Rheumatoid; Childhood Arthritides; Childhood Arthritis; Chronic Arthritis, Juvenile; Idiopathic Arthritis, Juvenile; Juvenile Arthritis; Juvenile Chronic Arthritis; Juvenile Idiopathic Arthritis; Juvenile Rheumatoid Arthritis; Rheumatoid Arthritis, Juvenile
SNOMED CT: Juvenile idiopathic arthritis (410502007); Juvenile rheumatoid arthritis (410502007); JIA - juvenile idiopathic arthritis (410502007)
 
Monarch Initiative: MONDO:0011429
OMIM®: 613409; 618795
Orphanet: ORPHA92

Definition

A rare, heterogeneous group of rheumatologic diseases characterized by arthritis which has an onset before 16 years of age, persists for more than 6 weeks, and is of unknown origin. [from ORDO]

Clinical features

From HPO
Thrombocytosis
MedGen UID:
163397
•Concept ID:
C0836924
•
Disease or Syndrome
Increased numbers of platelets in the peripheral blood.
See: Feature record | Search on this feature
Leukocytosis
MedGen UID:
9736
•Concept ID:
C0023518
•
Disease or Syndrome
An abnormal increase in the number of leukocytes in the blood.
See: Feature record | Search on this feature
Antinuclear antibody positivity
MedGen UID:
101792
•Concept ID:
C0151480
•
Laboratory or Test Result
The presence of autoantibodies in the serum that react against nuclei or nuclear components.
See: Search on this feature
Skin rash
MedGen UID:
1830322
•Concept ID:
C5779628
•
Sign or Symptom
A red eruption of the skin.
See: Feature record | Search on this feature
Periodic fever
MedGen UID:
798670
•Concept ID:
C0015974
•
Disease or Syndrome
Episodic fever that recurs at regular intervals.
See: Feature record | Search on this feature
Quotidian fever
MedGen UID:
1708317
•Concept ID:
C2219980
•
Sign or Symptom
Fever that occurs at daily intervals.
See: Feature record | Search on this feature
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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

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
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
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
Macrophage Activation Syndrome.
Ravelli A, Davì S, Minoia F, Martini A, Cron RQ
Hematol Oncol Clin North Am 2015 Oct;29(5):927-41. Epub 2015 Aug 25 doi: 10.1016/j.hoc.2015.06.010. PMID: 26461152
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

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
Anterior uveitis.
Gueudry J, Muraine M
J Fr Ophtalmol 2018 Jan;41(1):e11-e21. Epub 2017 Dec 29 doi: 10.1016/j.jfo.2017.11.003. PMID: 29290458
Juvenile Idiopathic Arthritis.
Barut K, Adrovic A, Şahin S, Kasapçopur Ö
Balkan Med J 2017 Apr 5;34(2):90-101. doi: 10.4274/balkanmedj.2017.0111. PMID: 28418334Free PMC Article
Macrophage Activation Syndrome.
Ravelli A, Davì S, Minoia F, Martini A, Cron RQ
Hematol Oncol Clin North Am 2015 Oct;29(5):927-41. Epub 2015 Aug 25 doi: 10.1016/j.hoc.2015.06.010. PMID: 26461152
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

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
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
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
Systemic Juvenile Idiopathic Arthritis.
Lee JJY, Schneider R
Pediatr Clin North Am 2018 Aug;65(4):691-709. doi: 10.1016/j.pcl.2018.04.005. PMID: 30031494
Juvenile Idiopathic Arthritis.
Barut K, Adrovic A, Şahin S, Kasapçopur Ö
Balkan Med J 2017 Apr 5;34(2):90-101. doi: 10.4274/balkanmedj.2017.0111. PMID: 28418334Free 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
The effects of TNF-alpha inhibitor therapy on the incidence of infection in JIA children: a meta-analysis.
Nagy A, Mátrai P, Hegyi P, Alizadeh H, Bajor J, Czopf L, Gyöngyi Z, Kiss Z, Márta K, Simon M, Szilágyi ÁL, Veres G, Mosdósi B
Pediatr Rheumatol Online J 2019 Jan 18;17(1):4. doi: 10.1186/s12969-019-0305-x. PMID: 30658717Free PMC Article
Relapse of Juvenile Idiopathic Arthritis-Associated Uveitis after Discontinuation of Immunomodulatory Therapy.
Acharya NR, Patel S, Homayounfar G, Enanoria WTA, Shakoor A, Chakrabarti A, Goldstein DA
Ocul Immunol Inflamm 2019;27(4):686-692. Epub 2018 Feb 16 doi: 10.1080/09273948.2018.1424341. PMID: 29451845Free PMC Article
Foveal Serous Retinal Detachment in Juvenile Idiopathic Arthritis-associated Uveitis.
Liang F, Terrada C, Ducos de Lahitte G, Quartier P, Lehoang P, Thorne JE, Bodaghi B
Ocul Immunol Inflamm 2016 Aug;24(4):386-91. Epub 2015 Jul 14 doi: 10.3109/09273948.2015.1012297. PMID: 26171661
What is the background incidence of malignancy in children with rheumatic disease?
Mannion ML, Beukelman T
Curr Rheumatol Rep 2013 Mar;15(3):310. doi: 10.1007/s11926-012-0310-2. PMID: 23378144

Clinical prediction guides

Imaging of Juvenile Idiopathic Arthritis.
Hemke R, Herregods N, Jaremko JL, Maas M
Radiol Clin North Am 2024 Sep;62(5):889-902. Epub 2024 Mar 22 doi: 10.1016/j.rcl.2024.02.009. PMID: 39059979
Widefield Fundus Fluorescein Angiography Features of Uveitis Associated with Juvenile Idiopathic Arthritis.
Tripathy K, Ying H, Maldonado Cerda A, Filipowicz A, Kaya M, Seymen Z, Anesi SD, Chang PY, Foster CS
Ocul Immunol Inflamm 2022 May 19;30(4):829-838. Epub 2020 Dec 2 doi: 10.1080/09273948.2020.1834586. PMID: 33264037
Shoulder arthroplasty for juvenile idiopathic arthritis.
Madanipour S, Prinja A, Lee M, Rashid A
J Orthop Surg (Hong Kong) 2020 Jan-Apr;28(1):2309499019890615. doi: 10.1177/2309499019890615. PMID: 31916484
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
Imaging of Juvenile Idiopathic Arthritis.
Dimitriou C, Boitsios G, Badot V, Lê PQ, Goffin L, Simoni P
Radiol Clin North Am 2017 Sep;55(5):1071-1083. Epub 2017 Jun 29 doi: 10.1016/j.rcl.2017.04.011. PMID: 28774449

Recent systematic reviews

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 Rheumatol 2022 Apr;74(4):553-569. Epub 2022 Mar 1 doi: 10.1002/art.42037. PMID: 35233993Free PMC Article
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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