Juvenile Idiopathic Arthritis Clinical Presentation

  • Author: David D Sherry, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Dec 8, 2011
 

History

Arthritis must be present for 6 weeks before the diagnosis of juvenile idiopathic arthritis (JIA) can be made. Disease onset is either insidious or abrupt, with morning stiffness or gelling phenomenon (ie, stiffness after long periods of sitting or inactivity) being a frequent complaint and arthralgia occurring during the day. A morning limp that improves with time may be noted, and a toddler may no longer stand in the crib in the morning or after naps.

Complaints of joint pain may not be predominant in the patients’ history, however; children often stop using joints normally (eg, develop contractures of joints, decreased wrist range, limp) rather than complain of pain. Up to a quarter of children with oligoarticular JIA have no pain.

Individuals with JIA may have a history of school absences, and their ability to participate in physical education classes reflects the severity of the disease or acute flares.

Systemic-onset JIA is characterized by spiking fevers, typically occurring once or twice each day, at about the same time of day, with temperature returning to normal or below normal. The fever pattern is very useful because infections, Kawasaki disease, and malignancy usually do not have such a predictable pattern.

Systemic-onset JIA is usually accompanied by an evanescent rash (lasting a few hours), which is typically nonpruritic, macular, and salmon colored on the trunk and extremities. Occasionally, the rash is extremely pruritic and resistant to antihistamine treatment.

Children with psoriatic arthritis may have typical psoriasis but dermatological manifestations may be subtle; careful attention should be paid to looking for nail pits. Dactylitis is characteristic of psoriatic arthritis.

Enthesitis-related arthritis frequently presents as evening and post-exercise pain. Attention should be given to buttock pain and back pain that improves with activity (inflammatory back pain). These children cannot lie in bed all morning but have to get up due to back pain.

Next

Physical Examination

JIA is a clinical diagnosis. A complete physical examination is critical for the diagnosis. Physical findings are important to provide criteria for diagnosis and to detect abnormalities suggestive of alternative etiologies. The diagnosis of JIA is based on the physical finding of arthritis in at least 1 joint that has persisted for at least 6 weeks, with other causes excluded, in an individual younger than 16 years.

No diagnostic serologic tests for JIA are recognized, aside from rheumatoid factor assay for subclassification of polyarticular disease. Other tests, such as antinuclear antibody and HLA-B27 assays, may help further define diagnosis and risk of complications.

Arthritis is defined as either intra-articular swelling on examination or as limitation of joint motion in association with pain, warmth, or erythema of the joint. The hips, temporomandibular joint, and small joints in the spine do not demonstrate swelling when affected by synovitis but demonstrate the combination of loss of motion and pain. The physical findings in JIA are a reflection of the extent of joint involvement.

In synovitis, in which there is synovial proliferation and an increase in joint volume, the joint is held in a position of maximum comfort. Limbs with synovitis are generally held in flexion. Range of motion often is limited only at the extremes.

In synovitis, the fingers may appear swollen, and the range of motion becomes painful. The wrist goes into flexion. In the knee, the parapatellar fossae often are obliterated, and a doughy synovium may be palpable. A soft, boggy swelling is appreciated in the popliteal fossa.

The hip is held in an attitude of flexion, abduction, and external rotation. Attempted range of motion will be painful to a varying degree. Guarding is an early sign of synovitis.

Cutaneous erythema is extremely rare in JIA. Its presence should alert one to look for another diagnosis.

Previous
Next

Systemic-Onset Juvenile Idiopathic Arthritis

A definite diagnosis of systemic-onset JIA must await the development of arthritis. This may occur at onset of the fever and rash or may lag by months or, rarely, years.

Physical examination findings include the following:

  • The child appears systemically ill
  • Arthralgia is often present
  • The child may have generalized myalgia
  • Evanescent, salmon-pink, macular rash (often linear) is found, predominantly on the trunk and the extremities; this rash, seen in the image below, is associated with fever spikes Systemic juvenile idiopathic arthritis (JIA) rash.Systemic juvenile idiopathic arthritis (JIA) rash.
  • Hepatosplenomegaly is often present
  • Lymphadenopathy is sometimes present, especially the axillary lymph nodes
  • Muscle tenderness to palpation may be observed
  • Serositis, including pleural and pericardial effusions, may be present, as is noted in the image belowChild with pericardial effusion due to systemic onChild with pericardial effusion due to systemic onset juvenile idiopathic arthritis (JIA).
  • Chest pain or shortness of breath may be a sign of pericarditis or pleuritis
  • Friction rub may occur in pericarditis but can be absent with a large pericardial effusion
  • S3, basilar rales, and hepatomegaly suggestive of heart failure may rarely be observed when myocarditis occurs in individuals with systemic-onset JIA
Previous
Next

Oligoarticular Juvenile Idiopathic Arthritis

Characteristics of oligoarticular JIA include the following:

  • In individuals with oligoarticular JIA, 4 or fewer joints (and in many cases, only 1 joint) are affected
  • Large, weight-bearing joints, such as the knees and ankles, are typically affected, as seen in the image belowEighteen-month-old girl with arthritis in her righEighteen-month-old girl with arthritis in her right knee. Note the flexion contracture of that knee.
  • Children appear to be well, despite ambulating with a limp
  • In cases of asymmetrical arthritis, chronic inflammation-related hyperemia in a knee or ankle may lead to overgrowth of that limb with subsequent leg-length discrepancy
  • Muscle atrophy, often of extensor muscles (eg, vastus lateralis, quadriceps when the knee is affected), may occur
  • Flexion contractures in the knees and, less commonly, the wrists are found

Involvement of a few small joints in the hands is atypical and suggests eventual development of polyarticular JIA or psoriatic arthritis. Dactylitis, or diffuse tenosynovitis of a finger or toe, also called a "sausage digit," is more typical of psoriatic arthritis or enthesitis-related arthritis.

Anterior uveitis (seen in the image below) is present in as many as 20% of children with oligoarticular and polyarticular JIA, especially those who are antinuclear antibody (ANA) positive. The uveitis is typically asymptomatic at onset and must be screened for with an ophthalmologic slit lamp examination.

Generally, children who were 6 years of age or younger at onset (especially of oligoarticular and psoriatic arthritis) and have a positive ANA test are screened by slit lamp exam every 3 months for 4 years or more and then every 6 months until at least 7 years after diagnosis.[14] Thereafter they are screened yearly for life.

Children who are at lesser risk (ie, have polyarticular disease and are ANA negative), are screened every 6 months for 7 years and then yearly. Children with systemic JIA are at very low risk and are screened yearly.

Acute anterior uveitis is one of the diagnostic criteria for enthesitis-related arthritis. These children with are screened initially and if symptomatic.

Older children with RF-positive polyarticular JIA should probably be screened yearly. There are few data on these children regarding their risk for uveitis.

Sequelae of chronic anterior uveitis. Note the posSequelae 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.
Previous
Next

Polyarticular Juvenile Idiopathic Arthritis

In polyarticular juvenile idiopathic arthritis, 5 or more joints are affected in the first 6 months after disease onset, weight-bearing joints are affected, rheumatoid nodules may be seen in patients with RF-positive disease, and symmetrical involvement of small joints in the hands is often found, as seen in the images below.

Patient with active polyarticular arthritis. Note 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 polyaWrist 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) effClose-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. LonPatient 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 inaHand 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.

Decreased extension of the cervical spine is often asymptomatic. It is indicative of arthritis of the cervical spine and can lead to subluxation, typically of the C2 vertebra on C3. Fusion of the posterior elements of the vertebra may occur. (See the image below.)

Flexion and extension views of C-spine in child wiFlexion and extension views of C-spine in child with poorly controlled polyarticular juvenile idiopathic arthritis (JIA).

Arthritis of the temporal-mandibular joint (TMJ) may lead to micrognathia. TMJ arthritis is typically asymptomatic; decreased mouth aperture, lateral deviation of the jaw gait, or auscultatory abnormalities over the TMJ are signs of underlying arthritis (see the image below).

Temporal-mandibular joint (TMJ) MRI postgadoliniumTemporal-mandibular joint (TMJ) MRI postgadolinium infusion. Abnormal increased uptake indicative of synovitis in child with polyarticular juvenile idiopathic arthritis (JIA).
Previous
Next

Psoriatic Arthritis

Psoriatic arthritis in children is usually mild. Onset of arthritis precedes that of psoriasis in approximately half of children.

Characteristics of psoriatic arthritis include the following:

  • Monoarticular arthritis (50% of children)
  • DIP joint involvement (50%)
  • Tenosynovitis (30%)
  • Nail involvement(71%) - pitting is the most common but least specific finding
  • Disordered bone growth with resultant shortening (47%)
  • Sacroiliitis (28%)

For further discussion of this disorder, see Psoriatic Arthritis.

Previous
Next

Enthesitis-Related Arthritis

Enthesitis-related arthritis, or pediatric spondyloarthropathy, is characterized by periods of inflammation of tendons and ligaments, particularly at the area of insertion into bone (entheses). Often, children and adolescents with spondyloarthropathy present with arthritis, making the distinction between subtypes difficult. Furthermore, children occasionally develop a disease that appears to be a combination of the 2 diseases.

Pain and tenderness at the enthesis is the most common manifestation, but swelling may also be seen. In children, the initial manifestations involve mainly the peripheral joints (eg, dactylitis) with asymmetric oligoarticular arthritis of the lower limbs; axial involvement (eg, sacroiliitis) tends to appear later in the disease course.[11]

Diagnostic criteria for enthesitis-related JIA are the presence of both arthritis and enthesitis, or the presence of arthritis or enthesitis along with any 2 of the following 5 manifestations[15] :

  • Sacroiliac tenderness and/or inflammatory lumbosacral pain
  • Positive human leukocyte antigen B27 (HLA-B27) test
  • Onset of arthritis in a male 6 years old or older
  • Acute symptomatic anterior uveitis
  • Presence in a first-degree relative of ankylosing spondylitis, enthesitis-related arthritis, inflammatory bowel disease with sacroiliitis, reactive arthritis, or acute anterior uveitis

Although enthesitis can be observed in persons with oligoarticular and polyarticular JIA, the eventual development of arthritis into a predominant enthesitis is more characteristic of spondyloarthropathy. The radiographic changes observed in adults (eg, sclerosis of the sacroiliac joints, bamboo spine) are rare in childhood and adolescence.

Previous
Next

Undifferentiated Arthritis

Undifferentiated JIA is diagnosed if the patient’s manifestations either do not fulfill the criteria for any one category or fulfill the criteria for more than one.

Most often, children in the latter category fulfill the criteria for polyarticular RF-negative JIA and either enthesitis-related JIA or psoriatic JIA.[15]

Previous
Next

Complications of Disease

Systemic-onset juvenile idiopathic arthritis

The complications that may occur in systemic-onset JIA are pericarditis, hemolytic anemia, macrophage activation syndrome (MAS), and endarteritis. Patients with pericarditis often present with orthopnea and respond to intravenous (IV) corticosteroid treatment.

MAS is a rare, but important, complication, in which all 3 bloodlines become rapidly decreased. Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels and markedly elevated ferritin levels are hallmarks. Hypotension, central nervous system (CNS) disease, and marked hepatosplenomegaly may be noted as complications of a release of massive amounts of cytokines.

Children with limited arthritis

Complications of oligoarticular JIA and psoriatic arthritis include joint contractures, uveitis, and leg-length discrepancy. Uveitis is almost always asymptomatic and more frequent in young girls who have positive levels of antinuclear antibody. Evaluation with a slit-lamp every 4 months by a pediatric ophthalmologist can detect early disease to prevent permanent eye damage and even blindness.

Leg-length discrepancy may complicate unilateral knee involvement. In young children, it may result from neovascularization of growth plates, so the involved limb is longer. In early puberty, unilateral arthritis can lead to premature fusion of the epiphysis, in which case the short limb is on the affected side. The problem may not be detected in patients with a knee flexion contracture until the contracture is corrected. Both flexion contractures and leg-length discrepancies are much less frequent with early intervention.

Children with widespread arthritis

Complications of polyarticular JIA include skeletal abnormalities such as increased size of epiphyses, accelerated bone age, narrowed joint spaces, swan-neck and/or boutonniere deformities, joint subluxation, and cervical spine involvement.

Difficulty extending the spine may create a problem for intubation prior to surgery, so anesthesiologists need to be informed of the patient's diagnosis. Cervical spine radiography (in flexion and extension) may help to screen for potential difficulties during induction of anesthesia. High-level subluxation is a potential complication.

Enthesitis-related arthritis

Complications of enthesitis-elated arthritis are rare but can include restrictive lung disease and aortic insufficiency. These children are at risk for symptomatic iritis with acute photophobia and conjunctivitis but only rarely does it lead to visual impairment.

Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

David D Sherry, MD  Director, Clinical Rheumatology, Attending Physician, Pain Management, 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 and 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: Abbott Grant/research funds clincal trial; Pfizer Grant/research funds clinical trial; Centacor Grant/research funds clinical trial

Murali Poduval, MBBS, MS, DNB  Additional Professor in 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: Association of Medical Consultants of Mumbai, Bombay Orthopedic Society, Indian Orthopedic Association, and Indian Society of Hip and Knee Surgeons

Disclosure: Nothing to disclose.

Atul R S Bhaskar, MBBS, MCh, MS, FRCS, DNB(Orth)  Assistant Professor, Department of Orthopedics, K J Somaiya Medical College Hospital; Consulting Staff, Department of Orthopedic Surgery, BSES MG Hospital, India

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Barry L Myones, MD  Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital

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, and Texas Medical Association

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

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 Association for the History of Medicine, American College of Sports Medicine, American Orthopaedic Society for Sports Medicine, Massachusetts Medical Society, and Pediatric Orthopaedic Society of North America

Disclosure: Smith & Nephew Endoscopy Consulting fee Consulting; EBI Biomet Consulting fee Consulting; OrthoPediatrics Consulting fee Consulting; Pivot Medical Stock Consulting; pediped Consulting fee Consulting; WB Saunders Royalty None; Fixes-4-Kids Consulting

Dennis P Grogan, MD  Clinical Professor, Department of Orthopedic Surgery, University of South Florida College of Medicine; Chief of Staff, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa

Dennis P Grogan, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, American Orthopaedic Foot and Ankle Society, Eastern Orthopaedic Association, Irish American Orthopaedic Society, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society

Disclosure: Nothing to disclose.

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, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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

  2. 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. Mar 2009;19(3):693-700. [Medline].

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

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

  5. 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. Jan 2009;60(1):251-7. [Medline]. [Full Text].

  6. 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. Mar 2011;56(3):196-9. [Medline].

  7. 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. Apr 2001;44(4):794-801. [Medline].

  8. 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. Dec 2008;58(12):3924-31. [Medline]. [Full Text].

  9. Ayaz NA, Ozen S, Bilginer Y, Ergüven M, Taskiran E, Yilmaz E, et al. MEFV mutations in systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford). Jan 2009;48(1):23-5. [Medline].

  10. 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. Jan 2008;58(1):15-25. [Medline]. [Full Text].

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

  12. Sullivan DB, Cassidy JT, Petty RE. Pathogenic implications of age of onset in juvenile rheumatoid arthritis. Arthritis Rheum. May-Jun 1975;18(3):251-5. [Medline].

  13. Simard JF, Neovius M, Hagelberg S, Askling J. Juvenile idiopathic arthritis and risk of cancer: a nationwide cohort study. Arthritis Rheum. Dec 2010;62(12):3776-82. [Medline].

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

  15. Lovell DJ. Juvenile Idiopathic Arthritis: Clinical Features. In: Kippel JH, Stone JH, Crofford LJ, White PH, Eds. Primer on the Rheumatic Diseases. 13th Ed. Springer Science, New York: 2008.

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

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

  18. 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. Aug 2008;35(8):1668-75. [Medline].

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

  20. 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. Jun 2007;37(6):535-43. [Medline].

  21. 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). Apr 2007;46(4):699-702. [Medline].

  22. 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. Apr 2008;28(6):573-8. [Medline].

  23. American College of Rheumatology, Subcommittee on Rheumatoid Arthritis Guidelines. Guidelines for the management of rheumatoid arthritis: 2002 Update. Arthritis Rheum. Feb 2002;46(2):328-46. [Medline].

  24. 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). Apr 2011;63(4):465-82. [Medline].

  25. 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. European League Against Rheumatism (EULAR). [Full Text].

  26. Otten MH, Prince FH, Armbrust W, et al. Factors associated with treatment response to etanercept in juvenile idiopathic arthritis. JAMA. Dec 7 2011;306(21):2340-7. [Medline].

  27. 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.

  28. 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. Jan 1997;79(1):44-52. [Medline].

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

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

  31. 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. Apr 2006;77(2):242-50. [Medline].

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

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

  34. [Best Evidence] 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. Oct 2008;67(10):1412-6. [Medline].

  35. 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. Sep 1997;79(5):792-5. [Medline].

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

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

  38. 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. Mar 2008;128(3):249-54. [Medline].

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

  40. 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. Jun 2006;447:85-91. [Medline].

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

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

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

  44. 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. Apr 2007;78(2):206-10. [Medline].

  45. Yun AG, Martin S, Zurakowski D, Scott R. Bipolar hemiarthroplasty in juvenile rheumatoid arthritis: long-term survivorship and outcomes. J Arthroplasty. Dec 2002;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
ClassificationACR(1977)ILAR (1997)
NomenclatureJuvenile rheumatoid arthritisJuvenile idiopathic arthritis
Minimum duration≥6 wk≥6 wk
Age at onset< 16 y< 16 y
≤ 4 joints in first 6 mo after presentationPauciarticular juvenile rheumatoid arthritisOligoarticular 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 presentationPolyarticular juvenile rheumatoid arthritisPolyarticular juvenile idiopathic arthritis-rheumatoid factor negative



Polyarticular juvenile arthritis-rheumatoid factor positive



Fever, rash, arthritisSystemic juvenile rheumatoid arthritisSystemic juvenile idiopathic arthritis
Other categories includedExclusion of other formsPsoriatic 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 spondylitisNoYes
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.