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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.
Leukocytosis
MedGen UID:
9736
Concept ID:
C0023518
Disease or Syndrome
An abnormal increase in the number of leukocytes in the blood.
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.
Skin rash
MedGen UID:
1830322
Concept ID:
C5779628
Sign or Symptom
A red eruption of the skin.
Periodic fever
MedGen UID:
798670
Concept ID:
C0015974
Disease or Syndrome
Episodic fever that recurs at regular intervals.
Quotidian fever
MedGen UID:
1708317
Concept ID:
C2219980
Sign or Symptom
Fever that occurs at daily intervals.

Professional guidelines

PubMed

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
Mimura T, Kondo Y, Ohta A, Iwamoto M, Ota A, Okamoto N, Kawaguchi Y, Kono H, Takasaki Y, Takei S, Nishimoto N, Fujimoto M, Asanuma YF, Mimori A, Okiyama N, Kaneko S, Takahashi H, Yokosawa M, Sumida T
Mod Rheumatol 2018 Sep;28(5):736-757. Epub 2018 May 9 doi: 10.1080/14397595.2018.1465633. PMID: 29651907

Recent clinical studies

Etiology

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
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
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, 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
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

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
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
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
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

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
McCurdy D, Parsa MF
Adv Pediatr 2021 Aug;68:143-170. doi: 10.1016/j.yapd.2021.05.014. PMID: 34243850
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
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

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
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
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
Crayne CB, Beukelman T
Pediatr Clin North Am 2018 Aug;65(4):657-674. doi: 10.1016/j.pcl.2018.03.005. PMID: 30031492
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

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
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
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
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
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

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
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
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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