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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

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
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
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

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
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
Research progress in drug therapy of juvenile idiopathic arthritis.
Zhao WJ, Deng JH, Li CF
World J Pediatr 2022 Jun;18(6):383-397. Epub 2022 Apr 1 doi: 10.1007/s12519-022-00530-8. PMID: 35364799
Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East.
Al-Mayouf SM, Al Mutairi M, Bouayed K, Habjoka S, Hadef D, Lotfy HM, Scott C, Sharif EM, Tahoun N
Pediatr Rheumatol Online J 2021 Dec 2;19(1):166. doi: 10.1186/s12969-021-00650-x. PMID: 34857004Free PMC Article
Juvenile Idiopathic Arthritis: Oligoarthritis and Polyarthritis.
Crayne CB, Beukelman T
Pediatr Clin North Am 2018 Aug;65(4):657-674. doi: 10.1016/j.pcl.2018.03.005. PMID: 30031492
Prevalence and incidence of juvenile idiopathic arthritis: a systematic review.
Thierry S, Fautrel B, Lemelle I, Guillemin F
Joint Bone Spine 2014 Mar;81(2):112-7. Epub 2013 Nov 6 doi: 10.1016/j.jbspin.2013.09.003. PMID: 24210707

Clinical prediction guides

Enthesitis-related arthritis: monitoring and specific tools.
Lassoued Ferjani H, Maatallah K, Miri S, Triki W, Nessib DB, Kaffel D, Hamdi W
J Pediatr (Rio J) 2022 May-Jun;98(3):223-229. Epub 2021 Sep 28 doi: 10.1016/j.jped.2021.08.002. PMID: 34597529Free PMC Article
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
Clinical outcome measures in juvenile idiopathic arthritis.
Consolaro A, Giancane G, Schiappapietra B, Davì S, Calandra S, Lanni S, Ravelli A
Pediatr Rheumatol Online J 2016 Apr 18;14(1):23. doi: 10.1186/s12969-016-0085-5. PMID: 27089922Free PMC Article

Recent systematic reviews

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations.
Stoustrup P, Resnick CM, Abramowicz S, Pedersen TK, Michelotti A, Küseler A, Koos B, Verna C, Nordal EB, Granquist EJ, Halbig JM, Kristensen KD, Kaban LB, Arvidsson LZ, Spiegel L, Stoll ML, Lerman MA, Glerup M, Defabianis P, Frid P, Alstergren P, Cron RQ, Ringold S, Nørholt SE, Peltomaki T, Larheim TA, Herlin T, Peacock ZS, Kellenberger CJ, Twilt M; Temporomandibular Joint Juvenile Arthritis Working Group
Arthritis Rheumatol 2023 Jan;75(1):4-14. Epub 2022 Nov 25 doi: 10.1002/art.42338. PMID: 36041065Free 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 Rheumatol 2022 Apr;74(4):553-569. Epub 2022 Mar 1 doi: 10.1002/art.42037. PMID: 35233993Free PMC Article
Exercise Therapy in Juvenile Idiopathic Arthritis: A Systematic Review and Meta-Analysis.
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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