Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Juvenile Idiopathic Arthritis

  • Author: David D Sherry, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Mar 03, 2016
 

Practice Essentials

Juvenile rheumatoid arthritis (JRA) is the most common chronic rheumatologic disease in children and is one of the most common chronic diseases of childhood (see the image below). The etiology is unknown, and the genetic component is complex, making clear distinctions between the various subtypes difficult. A new nomenclature, juvenile idiopathic arthritis (JIA), is being increasingly used to provide better definition of subgroups.

Eighteen-month-old girl with arthritis in her righ Eighteen-month-old girl with arthritis in her right knee. Note the flexion contracture of that knee.

Essential update: Children exposed to antibiotics may be at greater risk for juvenile arthritis

In a nested case–control study of 153 children with juvenile arthritis and 1,530 matched controls, researchers found that exposure to antibiotics during childhood significantly increased the risk for developing JIA (adjusted odds ratio = 2.6) in a dose-dependent manner. Compared with those with no exposure, the odds ratio for developing JIA was 3.1 for children exposed to one or two courses of antibiotics, and for those exposed to three to five courses the odds ratio was 3.8.[1]

The association between antibiotic exposure and JIA was similar for different classes of antibiotics. No association was found between exposure to nonbacterial antimicrobial agents and JIA. Adjustment for the number and type of infections and age at antibiotic exposure did not change the associations significantly.[1]

Signs and symptoms

History findings in children with JIA may include the following:

  • Arthritis present for at least 6 weeks before diagnosis (mandatory for diagnosis of JIA)
  • Either insidious or abrupt disease onset, often with morning stiffness or gelling phenomenon and arthralgia during the day
  • Complaints of joint pain or abnormal joint use
  • History of school absences or limited ability to participate in physical education classes
  • Spiking fevers occurring once or twice each day at about the same time of day
  • Evanescent rash on the trunk and extremities
  • Psoriasis or more subtle dermatologic manifestations

Physical findings are important to provide criteria for diagnosis and to detect abnormalities suggestive of alternative etiologies, as well as to indicate disease subtypes. Such findings include the following:

  • Arthritis: Defined either as intra-articular swelling on examination or as limitation of joint motion in association with pain, warmth, or erythema of the joint; physical findings in JIA reflect the extent of joint involvement
  • Synovitis: Characterized by synovial proliferation and increased joint volume; the joint is held in a position of maximum comfort, and range of motion often is limited only at the extremes

Types of JIA include the following:

  • Systemic-onset juvenile idiopathic arthritis
  • Oligoarticular juvenile idiopathic arthritis
  • Polyarticular juvenile idiopathic arthritis
  • Psoriatic arthritis
  • Enthesitis-related arthritis
  • Undifferentiated arthritis

See Clinical Presentation for more detail.

Diagnosis

Diagnosis of JIA is based on the history and physical examination findings. When physical findings do not document definite arthritis, further evaluation is warranted. Laboratory studies that may be considered include the following:

  • Inflammatory markers: Erythrocyte sedimentation rate (ESR) or CRP level
  • Complete blood count (CBC) and metabolic panel
  • Liver function tests and assessment of renal function with serum creatinine levels
  • Antinuclear antibody (ANA) testing
  • Rheumatoid factor (RF) and anti–cyclic citrullinated peptide (CCP) antibody
  • Additional studies: Total protein, albumin, fibrinogen, ferritin, D-dimer, angiotensin-converting enzyme (ACE), antistreptolysin 0 (AS0), anti-DNAse B, urinalysis

When only a single joint is affected, radiography is important to exclude other diseases. Basic radiographic changes in JIA include the following:

  • Soft tissue swelling
  • Osteopenia or osteoporosis
  • Joint-space narrowing
  • Bony erosions
  • Intra-articular bony ankylosis
  • Periosteitis
  • Growth disturbances
  • Epiphyseal compression fracture
  • Joint subluxation
  • Synovial cysts

Other imaging modalities that may be helpful include the following:

  • Computed tomography
  • Magnetic resonance imaging
  • Ultrasonography and echocardiography
  • Nuclear imaging

Other studies and procedures that may be considered include the following:

  • Dual-energy radiographic absorptiometry (DRA)
  • Arthrocentesis and synovial biopsy
  • Pericardiocentesis

See Workup for more detail.

Management

A team-based approach to the treatment of JIA can be helpful. Management may include 1 or all of the following areas:

  • Pharmacologic therapy with nonsteroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs), biologic agents, or intra-articular and oral corticosteroids
  • Psychosocial interventions
  • Measures to enhance school performance (eg, academic counseling)
  • Improved nutrition
  • Physical therapy
  • Occupational therapy

American College of Rheumatology (ACR) criteria for complete remission are as follows[2] :

  • No inflammatory joint pain
  • No morning stiffness
  • No fatigue
  • No synovitis
  • No progression of damage, as determined in sequential radiographic examinations
  • No elevation of the ESR and CRP level

The ACR recommends treatment approaches to JIA on the basis of the following 5 treatment groups[3] :

  • A history of arthritis in 4 or fewer joints
  • A history of arthritis in 5 or more joints
  • Active sacroiliac arthritis
  • Systemic arthritis without active arthritis
  • Systemic arthritis with active arthritis

Within each group, choice of therapy is guided by the severity of disease activity and the presence or absence of features indicating a poor prognosis.

Advances in medical treatment have reduced the need for surgical intervention. Procedures that may be considered in specific circumstances include the following:

  • Synovectomy
  • Osteotomy and arthrodesis
  • Hip and knee replacement

See Treatment and Medication for more detail.

Next

Background

Juvenile rheumatoid arthritis (JRA) is the most common chronic rheumatologic disease in children and is one of the most common chronic diseases of childhood. It represents a group of disorders that share the clinical manifestation of chronic joint inflammation.

The etiology is unknown, and the genetic component is complex, making clear distinctions between the various subtypes difficult. As a result, the various sets of classification criteria that have been recognized have different benefits and limitations. A new nomenclature, juvenile idiopathic arthritis (JIA), is being increasingly used in order to better define subgroups.

Go to Osteoarthritis, Pediatric Osteoarthritis, and Rheumatoid Arthritis for complete information on these topics.

Criteria and classification

Three groups have developed sets of criteria to classify children with arthritis: the American College of Rheumatology (ACR), the European League Against Rheumatism (EULAR), and the International League of Associations for Rheumatology (ILAR).[4, 5, 6]

The ACR criteria define juvenile rheumatoid arthritis (JRA) by age limit (< 16 y) and the duration of disease (>6 weeks). (See Table.) The organization recognizes the following 3 subtypes:

  • Polyarticular
  • Pauciarticular
  • Systemic

Other forms of childhood arthritis, such as juvenile ankylosing spondylitis and psoriatic arthritis, are classified under spondyloarthropathies.

The ILAR classification of JIA includes the following categories:

  • Systemic-onset JIA
  • Persistent or extended oligoarthritis
  • Rheumatoid factor (RF)–positive polyarthritis
  • RF-negative polyarthritis
  • Psoriatic JIA
  • Enthesitis-related arthritis
  • Undifferentiated - The disease does not meet criteria for any of the other subgroups, or it meets more than 1 criterion (and therefore could be classified in a number of subgroups).

The EULAR proposed the term juvenile chronic arthritis (JCA) for the heterogeneous group of disorders that manifest as juvenile arthritis. The diagnosis requires that the arthritis begins before age 16 years and lasts for at least 3 months. The EULAR criteria for JCA recognize the following subtypes, based on characteristics at onset:

  • Pauciarticular (1-4 joints)
  • Polyarticular (≥5 joints)
  • Presence of RF (2 positive tests at least 3 months apart)
  • Systemic onset with characteristic features
  • Positivity for rheumatoid factor
  • Juvenile ankylosing spondylitis
  • Juvenile psoriatic arthritis

This article will use the ILAR nomenclature unless differentiation is required between JIA and JRA or JCA.

Table. Comparison of Classification Criteria for Chronic Childhood Arthritis (Open Table in a new window)

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

 

Previous
Next

Etiology and Pathophysiology

The etiology and pathogenesis of JIA are not completely understood. Genetic susceptibility plays a major role, but there is significant overlap between loci associated with JIA and those associated with other autoimmune diseases.[7]

JIA is a genetically complex disorder in which multiple genes are important for disease onset and manifestations. The IL2RA/CD25 gene has been implicated as a JIA susceptibility locus, as has the VTCN1 gene.[8] Associations have been found between specific HLA alleles and clinical subtypes of JIA (eg, HLA-A(*)02:06 with susceptibility to JIA accompanied by uveitis, and HLA-DRB1(*)04:05 with polyarticular JIA, in a Japanese cohort).[9]  

A study by Ombrello examined the MHC locus in a large collection of systemic juvenile idiopathic arthritis patients and verified the relationship between the class II HLA region and systemic juvenile idiopathic arthritis, implicating adaptive immune molecules in the pathogenesis of the disease.[10]

Humoral and cell-mediated immunity are involved in the pathogenesis of JIA. T lymphocytes have a central role, releasing proinflammatory cytokines (eg, tumor necrosis factor–alpha [TNF-α], interleukin [IL]-6, IL-1) and favoring a type-1 helper T-lymphocyte response. A disordered interaction between type 1 and type 2 T-helper cells has been postulated.

Studies of T-cell receptor expression confirm recruitment of T-lymphocytes specific for synovial nonself antigens. Evidence for abnormalities in the humoral immune system include the increased presence of autoantibodies (especially antinuclear antibodies), increased serum immunoglobulins, the presence of circulating immune complexes, and complement activation.

Chronic inflammation of synovium is characterized by B-lymphocyte infiltration and expansion. Macrophages and T-cell invasion are associated with the release of cytokines, which evoke synoviocyte proliferation. A study by Scola et al found synovium to contain messenger ribonucleic acid (mRNA) for vascular endothelial growth factor and angiopoietin 1, as well as for their receptors, suggesting that induction of angiogenesis by products of lymphocytic infiltration may be involved in persistence of disease.[11]

Some pediatric rheumatologists view systemic-onset JIA as an autoinflammatory disorder, such as familial Mediterranean fever (FMF) or cryopyrin-associated periodic fever syndromes, rather than a subtype of JIA. This theory is supported by work demonstrating similar expression patterns of a phagocytic protein (S100A12) in systemic-onset JIA and FMF, as well as the same marked responsiveness to IL-1 receptor antagonists.[12]

FMF is associated with mutations in the MEFV gene; these mutations are associated with activation of the IL-1b pathway, resulting in inflammation. A study by Ayaz et al found an increased frequency of MEFV mutations in Turkish children who were diagnosed with systemic JIA[13] ; this study has not been replicated in other populations.

Previous
Next

Epidemiology

United States statistics

Approximately 300,000 children in the United States are estimated to have some type of arthritis. The incidence rate estimates for JIA range from 4-14 cases per 100,000 children annually; for JRA, the prevalence has ranged from 1.6 to 86.1 cases per 100,000.[14] These wide-ranging numbers are attributable to differing definitions and criteria for childhood arthritis; population differences, including environmental exposure and immunogenetic susceptibility; and difficulty in case ascertainment and lack of population based data.

International statistics

Worldwide, JIA appears to occur more frequently in certain populations (eg, indigenous peoples) from such disparate areas as British Columbia and Norway. A study in Sweden found the prevalence of JIA there to be similar to that in Minnesota, approximately 85 cases per 100,000 population, with an incidence of 11 cases per 100,000 population. A study from Germany found a prevalence rate of 20 cases per 100,000 population, with an incidence rate of 3.5 cases per 100,000 population.

Estimates from Norway include a prevalence rate of 148 cases per 100,000 population with an incidence rate of 22 cases per 100,000 population. The incidence of JIA in Japan has been reported to be low.

Disease-associated mortality for JIA is difficult to quantify, but it is estimated to be less than 1% in Europe and less than 0.5% in North America. Most deaths associated with JIA in Europe are related to amyloidosis, and most in the United States are related to infections.

The approximate frequencies of the various forms of JRA are as follows:

  • Oligoarticular - 30%
  • Polyarticular RF negative - 20%
  • Polyarticular RF positive – 5%
  • Systemic-onset – 5%
  • Psoriatic - 5%
  • Enthesitis Related – 25%
  • Undifferentiated – 10%

Sexual differences in frequency

Girls with an oligoarticular onset outnumber boys by a ratio of 3:1. In children with uveitis, the ratio of girls to boys is 5-6.6:1, and in children with polyarticular onset, girls outnumber boys by 2.8:1. In striking contrast, systemic-onset occurs with equal frequency in boys and girls. Boys outnumber girls with enthesitis-related arthritis.[15]

Age-related differences in frequency

Although JIA is defined as arthritis beginning before age 16 years, the age at onset is often much lower, with the highest frequency occurring in children aged 1-3 years.[16] This age distribution is most evident in girls with oligoarticular JIA and psoriatic arthritis.

Polyarticular RF-negative JIA has a biphasic peak of onset; the first is at a young age (1-4 y), similar to oligoarticular JIA, and the second peak is at age 6-12 years. RF-positive disease is more common in adolescents. Systemic-onset JIA is not characterized by a peak age of onset; it is spread across the childhood years. The usual age of onset of enthesitis-related arthritis is 10-12 years.[15]

Previous
Next

Prognosis

Advances in treatment over the last 20 years—especially the introduction of early use of intra-articular steroids, methotrexate, and biologic medications—have dramatically improved the prognosis for children with arthritis. Almost all children with JIA lead productive lives. However, many patients, particularly those with polyarticular disease, may have problems with active disease throughout adulthood, with sustained remission attained in a minority of patients.

Early hip or wrist involvement, symmetrical disease, the presence of RF, and prolonged active systemic disease have been associated with poor long-term outcomes. Compared with adults with RF-positive rheumatoid arthritis, however, children are at less risk for rheumatoid lung involvement and vasculitis. The anti – cyclic citrullinated peptide antibodies (CCP) antibodies test may be more specific than the RF test, but it is not as well studied in children.

Children with systemic-onset disease tend to either respond completely to medical therapy or develop a severe polyarticular course that tends to be refractory to medical treatment, with disease persisting into adulthood.

Most children with oligoarticular disease demonstrate eventual permanent remission, although a small number progress to persisting polyarticular disease.

Concern has been raised that the use of biologics may increase cancer risk among patients with JIA; however, lack of data on the baseline risk of cancer in this population has made it difficult to determine whether the concern is justified. A review of a large cohort of patients from the Swedish registry found an increased risk of cancer in patients who had not been on biologic therapies and had been diagnosed with JIA in the last 20 years. However, this risk was not found if the analysis was extended to patients diagnosed between 1969 and 1987.[17, 18]

The results of this study were not statistically significant. Nevertheless, they may have implications for interpretation of cancer signals in patients with JIA, particularly those who are on ongoing therapy with biologic agents, such as TNF-alpha inhibitors.

Previous
Next

Patient Education

Educating the patient, family, and school personnel (eg, classroom teachers, physical education teachers, nurses) about JIA and its presentation, treatment, and potential effects is continually necessary. Members of the pediatric rheumatology team in pediatric rheumatology clinics are the best educators about JIA. Another important source of information is the American Juvenile Arthritis Organization, a council of the Arthritis Foundation.

For patient education information, see the Arthritis Center, as well as Juvenile Rheumatoid Arthritis and Juvenile Rheumatoid Arthritis Treatment.

Previous
 
 
Contributor Information and Disclosures
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)

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.

Murali Poduval, MBBS, MS, DNB Associate Professor, Department of Orthopedic Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), India

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

Disclosure: Nothing to disclose.

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

Disclosure: Nothing to disclose.

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 Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, Texas Medical Association

Disclosure: Nothing to disclose.

References
  1. 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.

  2. 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. [Medline].

  3. 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. [Medline].

  4. Lamer S, Sebag GH. MRI and ultrasound in children with juvenile chronic arthritis. Eur J Radiol. 2000 Feb. 33(2):85-93. [Medline].

  5. 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. [Medline].

  6. Lee EY, Sundel RP, Kim S, Zurakowski D, Kleinman PK. MRI findings of juvenile psoriatic arthritis. Skeletal Radiol. 2008 Nov. 37(11):987-96. [Medline].

  7. Barton A, Worthington J. Genetic susceptibility to rheumatoid arthritis: an emerging picture. Arthritis Rheum. 2009 Oct 15. 61(10):1441-6. [Medline].

  8. 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. [Medline]. [Full Text].

  9. 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. [Medline].

  10. 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. [Medline].

  11. 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. [Medline].

  12. 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. [Medline]. [Full Text].

  13. 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. [Medline].

  14. 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. [Medline]. [Full Text].

  15. Orphanet. Enthesitis-related arthritis. Available at http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=85438.

  16. 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. [Medline].

  17. 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. [Medline].

  18. Ostring GT, Singh-Grewal D. Juvenile idiopathic arthritis in the new world of biologics. J Paediatr Child Health. 2013 May 6. [Medline].

  19. Leegaard A, Lomholt JJ, Thastum M, Herlin T. Decreased Pain Threshold in Juvenile Idiopathic Arthritis: A Cross-sectional Study. J Rheumatol. 2013 May 1. [Medline].

  20. Cassidy J, Kivlin J, Lindsley C, Nocton J. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics. 2006 May. 117(5):1843-5. [Medline].

  21. 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.

  22. 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. [Medline].

  23. Johnson K, Gardner-Medwin J. Childhood arthritis: classification and radiology. Clin Radiol. 2002 Jan. 57(1):47-58. [Medline].

  24. McHugh K, Gupta R, Murray K. Imaging in juvenile chronic arthritis. Imaging. 1999. 11:91-7:

  25. 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. [Medline].

  26. Gylys-Morin VM. MR imaging of pediatric musculoskeletal inflammatory and infectious disorders. Magn Reson Imaging Clin N Am. 1998 Aug. 6(3):537-59. [Medline].

  27. 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. [Medline].

  28. 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. [Medline].

  29. 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. [Medline].

  30. Tarp S, Amarilyo G, Foeldvari I, Christensen R, Woo JM, Cohen N, et al. Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis: a systematic review and meta-analysis of randomized trials. Rheumatology (Oxford). 2015 Nov 30. [Medline].

  31. Kelly JC. JIA Recommendations Updated: Use First-Line Anakinra. Medscape Medical News. Available at http://www.medscape.com/viewarticle/811699. Accessed: September 30, 2012.

  32. Ringold S, Weiss PF, Beukelman T, et al. 2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among Children Receiving Biologic Medications. Arthritis Rheum. Oct 2013. 65 (10):2499–2512.

  33. Brooks M. FDA Approves Tocilizumab for Polyarticular JIA. Medscape Medical News. Available at http://www.medscape.com/viewarticle/803478. Accessed: May 14, 2013.

  34. 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. [Medline].

  35. 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.

  36. 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. [Medline].

  37. 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.

  38. Lowes R. FDA approves Ilaris for rare juvenile arthritis. Medscape Medical News. May 10, 2013. [Full Text].

  39. 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. [Medline].

  40. 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. [Medline].

  41. 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.

  42. 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.

  43. Actemra (tocilizumab) prescribing information [package insert]. South San Francisco, CA: Genentech, Inc. April 2013. Available at [Full Text].

  44. Chang CY, Meyer RM, Reiff AO. Impact of Medication Withdrawal Method on Flare-free Survival in Patients with Juvenile Idiopathic Arthritis on Combination Therapy. Arthritis Care Res (Hoboken). 2014 Sep 12. [Medline].

  45. Chmell MJ, Scott RD, Thomas WH, Sledge CB. Total hip arthroplasty with cement for juvenile rheumatoid arthritis. Results at a minimum of ten years in patients less than thirty years old. J Bone Joint Surg Am. 1997 Jan. 79(1):44-52. [Medline].

  46. Coda A, Fowlie PW, Davidson JE, Walsh J, Carline T, Santos D. Foot orthoses in children with juvenile idiopathic arthritis: a randomised controlled trial. Arch Dis Child. 2014 Mar 17. [Medline].

  47. Dabov G, Perez EA. Miscellaneous nontraumatic disorders: rheumatoid arthritis. Canale ST, ed. Campbell's Operative Orthopaedics. 10th ed. St Louis, Mo: Mosby; 2003.

  48. Delf Witt J. Surgical intervention and sports medicine: surgery in children. Isenberg D, Maddison P, Woo P, et al, eds. Oxford Textbook of Rheumatology. New York, NY: Oxford University Press; 2004. 1220-8.

  49. Goodman A. Early aggressive therapy succeeds in children with arthritis. Medscape Medical News. November 12, 2013. [Full Text].

  50. Goodman SB, Oh KJ, Imrie S, Hwang K, Shegog M. Revision total hip arthroplasty in juvenile chronic arthritis: 17 revisions in 11 patients followed for 4-12 years. Acta Orthop. 2006 Apr. 77(2):242-50. [Medline].

  51. Hackethal V. Juvenile Arthritis: Pattern of Drug Withdrawal Affects Flare. Medscape Medical News. Oct 6 2014. [Full Text].

  52. Hanson V, Kornreich HK, Bernstein B, et al. subtypes of juvenile rheumatoid arthritis (correlations of age at onset, sex, and serologic factors). Arthritis Rheum. 20 (Suppl):. 48(1):184.

  53. Heijstek MW, Kamphuis S, Armbrust W, Swart J, Gorter S, de Vries LD, et al. Effects of the live attenuated measles-mumps-rubella booster vaccination on disease activity in patients with juvenile idiopathic arthritis: a randomized trial. JAMA. 2013 Jun 19. 309(23):2449-56. [Medline].

  54. Ilowite NT. Current treatment of juvenile rheumatoid arthritis. Pediatrics. 2002 Jan. 109(1):109-15. [Medline].

  55. Kapetanovic MC, Lindqvist E, Saxne T, Eberhardt K. Orthopaedic surgery in patients with rheumatoid arthritis over 20 years: prevalence and predictive factors of large joint replacement. Ann Rheum Dis. 2008 Oct. 67(10):1412-6. [Medline].

  56. Kelly JC. MMR booster shots safe, effective in juvenile arthritis. Medscape Medical News. June 18, 2013. [Full Text].

  57. Lehtimäki MY, Lehto MU, Kautiainen H, Savolainen HA, Hämäläinen MM. Survivorship of the Charnley total hip arthroplasty in juvenile chronic arthritis. A follow-up of 186 cases for 22 years. J Bone Joint Surg Br. 1997 Sep. 79(5):792-5. [Medline].

  58. Lybäck CO, Lehto MU, Hämäläinen MM, Belt EA. Patellar resurfacing reduces pain after TKA for juvenile rheumatoid arthritis. Clin Orthop Relat Res. 2004 Jun. 152-6. [Medline].

  59. McCullough CJ. Surgical management of the hip in juvenile chronic arthritis. Br J Rheumatol. 1994 Feb. 33(2):178-83. [Medline].

  60. Mulhall KJ, Saleh KJ, Thompson CA, Severson EP, Palmer DH. Results of bilateral combined hip and knee arthroplasty in very young patients with juvenile rheumatoid arthritis. Arch Orthop Trauma Surg. 2008 Mar. 128(3):249-54. [Medline].

  61. Odent T, Journeau P, Prieur AM, Touzet P, Pouliquen JC, Glorion C. Cementless hip arthroplasty in juvenile idiopathic arthritis. J Pediatr Orthop. 2005 Jul-Aug. 25(4):465-70. [Medline].

  62. Oh KJ, Imrie S, Hwang K, Ramachandran R, Shegog M, Goodman SB. Total hip arthroplasty using the miniature Anatomic Medullary Locking stem. Clin Orthop Relat Res. 2006 Jun. 447:85-91. [Medline].

  63. Palmer DH, Mulhall KJ, Thompson CA, Severson EP, Santos ER, Saleh KJ. Total knee arthroplasty in juvenile rheumatoid arthritis. J Bone Joint Surg Am. 2005 Jul. 87(7):1510-4. [Medline].

  64. Thomas A, Rojer D, Imrie S, Goodman SB. Cemented total knee arthroplasty in patients with juvenile rheumatoid arthritis. Clin Orthop Relat Res. 2005 Apr. 140-6. [Medline].

  65. Williams WW, McCullough CJ. Results of cemented total hip replacement in juvenile chronic arthritis. A radiological review. J Bone Joint Surg Br. 1993 Nov. 75(6):872-4. [Medline].

  66. Wroblewski BM, Siney PD, Fleming PA. Charnley low-frictional torque arthroplasty in young rheumatoid and juvenile rheumatoid arthritis: 292 hips followed for an average of 15 years. Acta Orthop. 2007 Apr. 78(2):206-10. [Medline].

  67. Yun AG, Martin S, Zurakowski D, Scott R. Bipolar hemiarthroplasty in juvenile rheumatoid arthritis: long-term survivorship and outcomes. J Arthroplasty. 2002 Dec. 17(8):978-86. [Medline].

 
Previous
Next
 
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.
Table. Comparison of Classification Criteria for Chronic Childhood Arthritis
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
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.