American College of Rheumatology, Subcommittee on Rheumatoid Arthritis Guidelines. Guidelines for the management of rheumatoid arthritis: 2002 Update. Arthritis Rheum. 2002 Feb. 46(2):328-46. [QxMD MEDLINE Link].
Beukelman T, Patkar NM, Saag KG, Tolleson-Rinehart S, Cron RQ, Dewitt EM, et al. 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: Initiation and safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis Care Res (Hoboken). 2011 Apr. 63(4):465-82. [QxMD MEDLINE Link].
Lamer S, Sebag GH. MRI and ultrasound in children with juvenile chronic arthritis. Eur J Radiol. 2000 Feb. 33(2):85-93. [QxMD MEDLINE Link].
Argyropoulou MI, Margariti PN, Karali A, Astrakas L, Alfandaki S, Kosta P, et al. Temporomandibular joint involvement in juvenile idiopathic arthritis: clinical predictors of magnetic resonance imaging signs. Eur Radiol. 2009 Mar. 19(3):693-700. [QxMD MEDLINE Link].
Lee EY, Sundel RP, Kim S, Zurakowski D, Kleinman PK. MRI findings of juvenile psoriatic arthritis. Skeletal Radiol. 2008 Nov. 37(11):987-96. [QxMD MEDLINE Link].
Barton A, Worthington J. Genetic susceptibility to rheumatoid arthritis: an emerging picture. Arthritis Rheum. 2009 Oct 15. 61(10):1441-6. [QxMD MEDLINE Link].
Hinks A, Ke X, Barton A, Eyre S, Bowes J, Worthington J, et al. Association of the IL2RA/CD25 gene with juvenile idiopathic arthritis. Arthritis Rheum. 2009 Jan. 60(1):251-7. [QxMD MEDLINE Link]. [Full Text].
Yanagimachi M, Miyamae T, Naruto T, Hara T, Kikuchi M, Hara R, et al. Association of HLA-A(*)02:06 and HLA-DRB1(*)04:05 with clinical subtypes of juvenile idiopathic arthritis. J Hum Genet. 2011 Mar. 56(3):196-9. [QxMD MEDLINE Link].
Ombrello MJ, Remmers EF, Tachmazidou I, et al. HLA-DRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis. Proc Natl Acad Sci U S A. 2015 Dec 29. 112 (52):15970-5. [QxMD MEDLINE Link].
Scola MP, Imagawa T, Boivin GP, Giannini EH, Glass DN, Hirsch R, et al. Expression of angiogenic factors in juvenile rheumatoid arthritis: correlation with revascularization of human synovium engrafted into SCID mice. Arthritis Rheum. 2001 Apr. 44(4):794-801. [QxMD MEDLINE Link].
Wittkowski H, Frosch M, Wulffraat N, Goldbach-Mansky R, Kallinich T, Kuemmerle-Deschner J, et al. S100A12 is a novel molecular marker differentiating systemic-onset juvenile idiopathic arthritis from other causes of fever of unknown origin. Arthritis Rheum. 2008 Dec. 58(12):3924-31. [QxMD MEDLINE Link]. [Full Text].
Ayaz NA, Ozen S, Bilginer Y, Ergüven M, Taskiran E, Yilmaz E, et al. MEFV mutations in systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford). 2009 Jan. 48(1):23-5. [QxMD MEDLINE Link].
Harrison P. Antibiotics in Children Increase Risk for Juvenile Arthritis. Medscape Medical News. Available at http://www.medscape.com/viewarticle/835110. Accessed: November 22, 2014.
Helmick CG, Felson DT, Lawrence RC, Gabriel S, Hirsch R, Kwoh CK, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008 Jan. 58(1):15-25. [QxMD MEDLINE Link]. [Full Text].
Orphanet. Enthesitis-related arthritis. Available at http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=85438.
Sullivan DB, Cassidy JT, Petty RE. Pathogenic implications of age of onset in juvenile rheumatoid arthritis. Arthritis Rheum. 1975 May-Jun. 18(3):251-5. [QxMD MEDLINE Link].
Simard JF, Neovius M, Hagelberg S, Askling J. Juvenile idiopathic arthritis and risk of cancer: a nationwide cohort study. Arthritis Rheum. 2010 Dec. 62(12):3776-82. [QxMD MEDLINE Link].
Ostring GT, Singh-Grewal D. Juvenile idiopathic arthritis in the new world of biologics. J Paediatr Child Health. 2013 Sep. 49 (9):E405-12. [QxMD MEDLINE Link].
Leegaard A, Lomholt JJ, Thastum M, Herlin T. Decreased pain threshold in juvenile idiopathic arthritis: a cross-sectional study. J Rheumatol. 2013 Jul. 40 (7):1212-7. [QxMD MEDLINE Link].
Cassidy J, Kivlin J, Lindsley C, Nocton J. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics. 2006 May. 117(5):1843-5. [QxMD MEDLINE Link].
Lovell DJ. Juvenile Idiopathic Arthritis: Clinical Features. Kippel JH, Stone JH, Crofford LJ, White PH, Eds. Primer on the Rheumatic Diseases. 13th Ed. Springer Science, New York: 2008.
Gerss J, Roth J, Holzinger D, Ruperto N, Wittkowski H, et al. Phagocyte-specific S100 proteins and high-sensitivity C reactive protein as biomarkers for a risk-adapted treatment to maintain remission in juvenile idiopathic arthritis: a comparative study. Ann Rheum Dis. 2012 Dec. 71(12):1991-7. [QxMD MEDLINE Link].
Johnson K, Gardner-Medwin J. Childhood arthritis: classification and radiology. Clin Radiol. 2002 Jan. 57(1):47-58. [QxMD MEDLINE Link].
McHugh K, Gupta R, Murray K. Imaging in juvenile chronic arthritis. Imaging. 1999. 11:91-7:
Pedersen TK, Küseler A, Gelineck J, Herlin T. A prospective study of magnetic resonance and radiographic imaging in relation to symptoms and clinical findings of the temporomandibular joint in children with juvenile idiopathic arthritis. J Rheumatol. 2008 Aug. 35(8):1668-75. [QxMD MEDLINE Link].
Gylys-Morin VM. MR imaging of pediatric musculoskeletal inflammatory and infectious disorders. Magn Reson Imaging Clin N Am. 1998 Aug. 6(3):537-59. [QxMD MEDLINE Link].
Workie DW, Graham TB, Laor T, Rajagopal A, O'Brien KJ, Bommer WA, et al. Quantitative MR characterization of disease activity in the knee in children with juvenile idiopathic arthritis: a longitudinal pilot study. Pediatr Radiol. 2007 Jun. 37(6):535-43. [QxMD MEDLINE Link].
Nistala K, Babar J, Johnson K, Campbell-Stokes P, Foster K, Ryder C, et al. Clinical assessment and core outcome variables are poor predictors of hip arthritis diagnosed by MRI in juvenile idiopathic arthritis. Rheumatology (Oxford). 2007 Apr. 46(4):699-702. [QxMD MEDLINE Link].
Shanmugavel C, Sodhi KS, Sandhu MS, Sidhu R, Singh S, Katariya S, et al. Role of power Doppler sonography in evaluation of therapeutic response of the knee in juvenile rheumatoid arthritis. Rheumatol Int. 2008 Apr. 28(6):573-8. [QxMD MEDLINE Link].
Tarp S, Amarilyo G, Foeldvari I, et al. Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis: a systematic review and meta-analysis of randomized trials. Rheumatology (Oxford). 2016 Apr. 55 (4):669-79. [QxMD MEDLINE Link].
[Guideline] Ringold S, Angeles-Han ST, Beukelman T, et al. 2019 American College of Rheumatology/Arthritis Foundation guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for non-systemic polyarthritis, sacroiliitis, and enthesitis. Arthritis Rheumatol. 2019 Jun. 71 (6):846-63. [QxMD MEDLINE Link]. [Full Text].
Brooks M. FDA Approves Tocilizumab for Polyarticular JIA. Medscape Medical News. Available at http://www.medscape.com/viewarticle/803478. Accessed: May 14, 2013.
Klotsche J, Niewerth M, Haas JP, Huppertz HI, Zink A, Horneff G, et al. Long-term safety of etanercept and adalimumab compared to methotrexate in patients with juvenile idiopathic arthritis (JIA). Ann Rheum Dis. 2015 Apr 29. [QxMD MEDLINE Link].
Boggs W. Drugs for Juvenile Idiopathic Arthritis Have ‘Acceptable’ Tolerability. Reuters Health Information. Available at http://www.medscape.com/viewarticle/844424. May 11, 2015; Accessed: September 11, 2015.
Horneff G, Seyger MMB, Arikan D, Kalabic J, Anderson JK, Lazar A, et al. Safety of Adalimumab in Pediatric Patients with Polyarticular Juvenile Idiopathic Arthritis, Enthesitis-Related Arthritis, Psoriasis, and Crohn's Disease. J Pediatr. 2018 Oct. 201:166-175.e3. [QxMD MEDLINE Link].
Xeljanz (tofacitinib) [package insert]. New York, NY: Pfizer Labs. 2020 September. Available at [Full Text].
De Benedetti F, Brunner HI, Ruperto N, Kenwright A, Wright S, Calvo I, et al. Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012 Dec 20. 367(25):2385-95. [QxMD MEDLINE Link].
Efficacy and safety of tocilizumab in patients with systemic Juvenile Idiopathic Arthritis (sJIA): 12-week data from the phase 3 TENDER trial. Abstract presented on June 18, 2010. Available at http://) http://www.roche.com/investors/ir_update/inv-update-2010-10-18.htm.
Lowes R. FDA approves Ilaris for rare juvenile arthritis. Medscape Medical News. May 10, 2013. [Full Text].
Ruperto N, Brunner HI, Quartier P, Constantin T, Wulffraat N, Horneff G, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012 Dec 20. 367(25):2396-406. [QxMD MEDLINE Link].
Otten MH, Prince FH, Armbrust W, et al. Factors associated with treatment response to etanercept in juvenile idiopathic arthritis. JAMA. 2011 Dec 7. 306(21):2340-7. [QxMD MEDLINE Link].
Janeczko L. Children With Juvenile Idiopathic Arthritis May Benefit From Fitted Foot Orthoses. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/823449. Accessed: April 14, 2014.
Constantin T, Foeldvari I, Anton J, et al. Consensus-based recommendations for the management of uveitis associated with juvenile idiopathic arthritis: the SHARE initiative. Ann Rheum Dis. 2018 Aug. 77 (8):1107-17. [QxMD MEDLINE Link].
Ramanan AV, Dick AD, Jones AP, McKay A, Williamson PR, Compeyrot-Lacassagne S, et al. Adalimumab plus Methotrexate for Uveitis in Juvenile Idiopathic Arthritis. N Engl J Med. 2017 Apr 27. 376 (17):1637-46. [QxMD MEDLINE Link].
American College of Rheumatology, Section on Pediatric Rheumatology. Position statement: guidelines for referral of children and adolescents to pediatric rheumatologists. Available at http://www.rheumatology.org/sections/pediatric/ped_referral.pdf. Accessed: November 11, 1997.
Funk RS, Balevic S, Cooper JC, Becker ML. Therapeutics: nonbiologics. In: Petty RE, Laxer RM, Lindsley CB, Wedderburn LR, Mellins ED, Fuhlbrigge RC, eds. Textbook of Pediatric Rheumatology. 8th ed. Philadelphia, PA: Elsevier; 2021. eTable 12-1.
Actemra (tocilizumab) prescribing information [package insert]. South San Francisco, CA: Genentech, Inc. April 2013. Available at [Full Text].

Media Gallery

Patient with active polyarticular arthritis. Note swelling (effusions) of all proximal interphalangeal (PIP) joints in addition to boney overgrowth. Also note lack of distal interphalangeal joint (DIP) involvement. The patient has interosseus muscle wasting (observed on the dorsum of the hands), and subluxation and ulnar deviation of the wrists are present. Image courtesy of Barry L. Myones, MD.
Wrist radiographs of the patient with active polyarticular arthritis shown in Media file 2. Note severe loss of cartilage in the intercarpal spaces and the radiocarpal space of the right wrist. A large erosion is present in the articular surface of the ulnar epiphysis. The view of the left wrist shows boney ankylosis involving the lateral 4 carpal bones with sparing of the pisiform. Erosions are present in the distal radius and ulna. Almost a loss of cartilage has occurred between the radius and ulna and the carpus. Narrowing of the carpal/metacarpal joints is present. Image courtesy of Barry L. Myones, MD.
Close-up of the proximal interphalangeal (PIP) effusions in the patient with active polyarthritis shown in Media files 2 and 3. Synovial thickening and effusion, as well as boney overgrowth, are present at the PIP joints bilaterally. Image courtesy of Barry L. Myones, MD.
Patient with inactive polyarticular arthritis. Long-term sequelae of polyarticular disease includes joint subluxation (note both wrists and thumbs), joint contractures (at proximal interphalangeal joints [PIPs] and distal interphalangeal joints [DIPs]), boney overgrowth (at all PIPs), and finger deformities (eg, swan-neck or boutonniere deformities). Image courtesy of Barry L. Myones, MD.
Hand and wrist radiographs of the patient with inactive polyarticular arthritis shown in Media file 5. Long-term sequelae of polyarticular disease includes periarticular osteopenia, generalized increase in the size of epiphyses, accelerated bone age, narrowed joint spaces (especially at the fourth and fifth proximal interphalangeal joints [PIPs] bilaterally), boutonniere deformities (at left third and fourth interphalangeal joints), and medial subluxation of the first metacarpophalangeal joints (MCPs) bilaterally. Flattening and erosion of the radial carpal articular surface is present in both wrists. Mild narrowing of the joint spaces exists at the carpometacarpal joints and intercarpal rows bilaterally, with sclerotic change of the intercarpal row (right > left). The trapezium and trapezoid may be fused bilaterally. Image courtesy of Barry L. Myones, MD.
Sequelae of chronic anterior uveitis. Note the posterior synechiae (weblike attachments of the pupillary margin to the anterior lens capsule) of the right eye secondary to chronic anterior uveitis. This patient has a positive antinuclear antibodies (ANAs) and initially had a pauciarticular course of her arthritis. She now has polyarticular involvement but no active uveitis. Image courtesy of Carlos A. Gonzales, MD.
One set of suggested algorithms for the treatment of patients with juvenile arthritis. This should not be considered dogmatic because treatment is not standardized and remains empiric and, at times, controversial.
Systemic juvenile idiopathic arthritis (JIA) rash.
Child with pericardial effusion due to systemic onset juvenile idiopathic arthritis (JIA).
Flexion and extension views of C-spine in child with poorly controlled polyarticular juvenile idiopathic arthritis (JIA).
Temporal-mandibular joint (TMJ) MRI postgadolinium infusion. Abnormal increased uptake indicative of synovitis in child with polyarticular juvenile idiopathic arthritis (JIA).
Eighteen-month-old girl with arthritis in her right knee. Note the flexion contracture of that knee.
Ankylosis in the cervical spine at several levels due to long-standing juvenile rheumatoid arthritis (also known as juvenile idiopathic arthritis).
Widespread osteopenia, carpal crowding (due to cartilage loss), and several erosions affecting the carpal bones and metacarpal heads in particular in a child with advanced juvenile rheumatoid arthritis (also known as juvenile idiopathic arthritis).
(A) T2-weighted MRI shows high signal in both hips, which may be due to hip effusions or synovitis. High signal intensity in the left femoral head indicates avascular necrosis. (B) Coronal fat-saturated gadolinium-enhanced T1-weighted MRI shows bilateral enhancement in the hips. This indicated bilateral active synovitis, which is most pronounced on the right. Because the image was obtained with fat saturation, the hyperintensity in both hips is pathologic, reflecting an inflamed pannus.

of 15

Author

David D Sherry, MD Chief, Rheumatology Section, Director, Amplified Musculoskeletal Pain Program, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania School of Medicine

David D Sherry, MD is a member of the following medical societies: American College of Rheumatology, American Pain Society

Disclosure: Nothing to disclose.

Coauthor(s)

Atul R S Bhaskar, MBBS, FRCS(Tr&Orth), FRCS(Glasg), MCh(Orth), DNB(Orth) Assistant Professor/Lecturer, Department of Orthopedics, K J Somaiya Medical College Hospital; Pediatric Orthopedic Surgeon, BSES Hospital; Pediatric Orthopedic Surgeon, Bombay Hospital, India

Atul R S Bhaskar, MBBS, FRCS(Tr&Orth), FRCS(Glasg), MCh(Orth), DNB(Orth) is a member of the following medical societies: Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Murali Poduval, MBBS, MS, DNB Orthopaedic Surgeon, Senior Consultant, and Subject Matter Expert, Tata Consultancy Services, Mumbai, India

Murali Poduval, MBBS, MS, DNB is a member of the following medical societies: Association of Medical Consultants of Mumbai, Bombay Orthopedic Society, Indian Orthopedic Association, Indian Society of Hip and Knee Surgeons

Disclosure: Nothing to disclose.

C Egla Rabinovich, MD, MPH Associate Professor and Co-Division Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center

C Egla Rabinovich, MD, MPH is a member of the following medical societies: American College of Rheumatology

Disclosure: Received grant/research funds from Abbott for clincal trial; Received grant/research funds from UCB for clinical trial; Received grant/research funds from Janssen for clinical trial; Received grant/research funds from Hoffmann-La Roche Inc. for clinical trial.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Mininder S Kocher, MD, MPH Associate Professor of Orthopedic Surgery, Harvard Medical School/Harvard School of Public Health; Associate Director, Division of Sports Medicine, Department of Orthopedic Surgery, Children's Hospital Boston

Mininder S Kocher, MD, MPH is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Sports Medicine, Pediatric Orthopaedic Society of North America, American Association for the History of Medicine, American Orthopaedic Society for Sports Medicine, Massachusetts Medical Society

Disclosure: Received consulting fee from Smith & Nephew Endoscopy for consulting; Received consulting fee from EBI Biomet for consulting; Received consulting fee from OrthoPediatrics for consulting; Received stock from Pivot Medical for consulting; Received consulting fee from pediped for consulting; Received royalty from WB Saunders for none; Received stock from Fixes-4-Kids for consulting.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Barry L Myones, MD Co-Chair, Task Force on Pediatric Antiphospholipid Syndrome

Barry L Myones, MD is a member of the following medical societies: American Association of Immunologists, American Heart Association, American Society for Microbiology, Clinical Immunology Society

Disclosure: Nothing to disclose.

Classification	ACR(1977)	ILAR (1997)
Nomenclature	Juvenile rheumatoid arthritis	Juvenile idiopathic arthritis
Minimum duration	≥6 wk	≥6 wk
Age at onset	< 16 y	< 16 y
≤ 4 joints in first 6 mo after presentation	Pauciarticular juvenile rheumatoid arthritis	Oligoarticular juvenile idiopathic arthritis: (A) Persistent < 4 joints for course of disease; (B) Extended >4 joints after 6 mo
>4 joints in first 6 mo after presentation	Polyarticular juvenile rheumatoid arthritis	Polyarticular juvenile idiopathic arthritis-rheumatoid factor negative Polyarticular juvenile arthritis-rheumatoid factor positive
Fever, rash, arthritis	Systemic juvenile rheumatoid arthritis	Systemic juvenile idiopathic arthritis
Other categories included	Exclusion of other forms	Psoriatic juvenile idiopathic arthritis Enthesitis-related arthritis Undifferentiated: (A) Fits no other category; (B) Fits more than 1 category
Inclusion of psoriatic arthritis, inflammatory bowel disease, juvenile ankylosing spondylitis	No	Yes

Juvenile Idiopathic Arthritis Questions & Answers

References

Tables

Contributor Information and Disclosures

What would you like to print?