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KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Synonyms
- Juvenile rheumatoid arthritis (JRA), juvenile chronic arthritis
Definitions
- Juvenile idiopathic arthritis (JIA): Synovial inflammation of unknown cause
- Diagnostic criteria: Arthritis begins < 16 years of age, ≥ 6 weeks of symptoms, & other conditions excluded (diagnosis of exclusion)
- Most widely used classification developed by International League of Associations for Rheumatology (ILAR)
- Systemic arthritis (sJIA), previously known as Still disease: 10-20%
- Arthritis of ≥ 1 joint + fever of ≥ 2-weeks duration with daily (quotidian) fever for at least 3 consecutive days
- Accompanied by ≥ 1 of following: Evanescent rash, hepatomegaly &/or splenomegaly, serositis (pericarditis, pleuritis, peritonitis), generalized lymphadenopathy
- Can be associated with interstitial lung disease, macrophage activation syndrome, amyloidosis
- Oligoarticular arthritis: Most common form of JIA (50%)
- Arthritis of < 5 joints in first 6 months of disease
- Persistent: < 5 joints affected during entire disease
- Extended: ≥ 5 joints affected after first 6 months
- Young girls
- Large joints: Knee, ankle, elbow
- Iridocyclitis in up to 30%
- Arthritis of < 5 joints in first 6 months of disease
- Polyarticular rheumatoid factor (RF)-positive arthritis
- Arthritis of ≥ 5 joints in first 6 months of disease
- ≥ 2 positive tests for RF at least 3 months apart within first 6 months of disease
- More commonly adolescent girls
- Typical: Symmetric, small joints of hands
- Polyarticular RF-negative arthritis
- Arthritis of ≥ 5 joints in first 6 months of disease
- No characteristic pattern
- Any age throughout childhood
- Psoriatic arthritis (< 15%)
- Arthritis & psoriasis or arthritis & ≥ 2 of following: Dactylitis ("sausage digit"), nail pitting or onycholysis, psoriasis in 1st-degree relative
- Enthesitis-related arthritis (< 7%)
- Arthritis & enthesitis, or
- Arthritis or enthesitis with ≥ 2 of following
- Sacroiliac joint tenderness &/or inflammatory lumbosacral pain
- Positive HLA-B27 antigen test
- Onset of arthritis in male ≥ 6 years old
- Symptomatic anterior uveitis
- Presence of 1st-degree relative with ankylosing spondylitis, enthesitis-related arthritis, inflammatory bowel disease with sacroiliitis, Reiter syndrome, or acute anterior uveitis
- Enthesitis is most commonly at Achilles, plantar fascia
- Undifferentiated arthritis
- Does not fulfill criteria for any category or fulfills criteria for multiple categories
- Systemic arthritis (sJIA), previously known as Still disease: 10-20%
IMAGING
General Features
Radiographic Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Jana M et al: Role of imaging in childhood arthritis. J Clin Rheumatol. ePub, 2021
- Ntoulia A et al: Contrast-enhanced ultrasound for musculoskeletal indications in children. Pediatr Radiol. ePub, 2021
- Pracoń G et al: Conventional radiography and ultrasound imaging of rheumatic diseases affecting the pediatric population. Semin Musculoskelet Radiol. 25(1):68-81, 2021
- Schiettecatte E et al: MR imaging of rheumatic diseases affecting the pediatric population. Semin Musculoskelet Radiol. 25(1):82-93, 2021
- Malattia C et al: Current status of MR imaging of juvenile idiopathic arthritis. Best Pract Res Clin Rheumatol. 101629, 2020
- Malattia C et al: Tocilizumab may slow radiographic progression in patients with systemic or polyarticular-course juvenile idiopathic arthritis: post hoc radiographic analysis from two randomized controlled trials. Arthritis Res Ther. 22(1):211, 2020
- Lee JJY et al: Systemic juvenile idiopathic arthritis. Pediatr Clin North Am. 65(4):691-709, 2018
- Hemke R et al: Magnetic resonance imaging (MRI) of the knee as an outcome measure in juvenile idiopathic arthritis: an OMERACT reliability study on MRI scales. J Rheumatol. 44(8):1224-30, 2017
- Nguyen JC et al: US evaluation of juvenile idiopathic arthritis and osteoarticular infection. Radiographics. 37(4):1181-201, 2017
- Avenarius DF et al: Erosion or normal variant? 4-year MRI follow-up of the wrists in healthy children. Pediatr Radiol. 46(3):322-30, 2016
- Kumar S: Systemic juvenile idiopathic arthritis: diagnosis and management. Indian J Pediatr. 83(4):322-7, 2016
- Naredo E et al: One year in review: ultrasound in arthritis. Clin Exp Rheumatol. 34(1):1-10, 2016
- Nusman CM et al: Contrast-enhanced MRI of the knee in children unaffected by clinical arthritis compared to clinically active juvenile idiopathic arthritis patients. Eur Radiol. 26(4):1141-8, 2016
- Viswanathan V et al: Management of children with juvenile idiopathic arthritis. Indian J Pediatr. 83(1):63-70, 2016
- Carrasco R: Juvenile idiopathic arthritis overview and involvement of the temporomandibular joint: prevalence, systemic therapy. Oral Maxillofac Surg Clin North Am. 27(1):1-10, 2015
- Ording Muller LS et al: The joints in juvenile idiopathic arthritis. Insights Imaging. 6(3):275-84, 2015
- Hemke R et al: One-year followup study on clinical findings and changes in magnetic resonance imaging-based disease activity scores in juvenile idiopathic arthritis. J Rheumatol. 41(1):119-27, 2014
- Hemke R et al: Reliability and responsiveness of the Juvenile Arthritis MRI Scoring (JAMRIS) system for the knee. Eur Radiol. 23(4):1075-83, 2013
- Hemke R et al: The diagnostic accuracy of unenhanced MRI in the assessment of joint abnormalities in juvenile idiopathic arthritis. Eur Radiol. 23(7):1998-2004, 2013
- Sheybani EF et al: Imaging of juvenile idiopathic arthritis: a multimodality approach. Radiographics. 33(5):1253-73, 2013
- Beresford MW: Juvenile idiopathic arthritis: new insights into classification, measures of outcome, and pharmacotherapy. Paediatr Drugs. 13(3):161-73, 2011
- Prakken B et al: Juvenile idiopathic arthritis. Lancet. 377(9783):2138-49, 2011
- Beresford MW et al: New advances in the management of juvenile idiopathic arthritis--2: the era of biologicals. Arch Dis Child Educ Pract Ed. 94(5):151-6, 2009
- Vastert SJ et al: Systemic JIA: new developments in the understanding of the pathophysiology and therapy. Best Pract Res Clin Rheumatol. 23(5):655-64, 2009
- McGhee JL et al: Clinical utility of antinuclear antibody tests in children. BMC Pediatr. 4(1):13, 2004
- Flato B et al: Prognostic factors in juvenile rheumatoid arthritis: a case-control study revealing early predictors and outcome after 14.9 years. J Rheumatol. 30(2):386-93, 2003
- Ramanan AV et al: Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. J Rheumatol. 30(2):401-3, 2003
- Cassidy JT et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum. 29(2):274-81, 1986
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Clinical Issues
TERMINOLOGY
Synonyms
- Juvenile rheumatoid arthritis (JRA), juvenile chronic arthritis
Definitions
- Juvenile idiopathic arthritis (JIA): Synovial inflammation of unknown cause
- Diagnostic criteria: Arthritis begins < 16 years of age, ≥ 6 weeks of symptoms, & other conditions excluded (diagnosis of exclusion)
- Most widely used classification developed by International League of Associations for Rheumatology (ILAR)
- Systemic arthritis (sJIA), previously known as Still disease: 10-20%
- Arthritis of ≥ 1 joint + fever of ≥ 2-weeks duration with daily (quotidian) fever for at least 3 consecutive days
- Accompanied by ≥ 1 of following: Evanescent rash, hepatomegaly &/or splenomegaly, serositis (pericarditis, pleuritis, peritonitis), generalized lymphadenopathy
- Can be associated with interstitial lung disease, macrophage activation syndrome, amyloidosis
- Oligoarticular arthritis: Most common form of JIA (50%)
- Arthritis of < 5 joints in first 6 months of disease
- Persistent: < 5 joints affected during entire disease
- Extended: ≥ 5 joints affected after first 6 months
- Young girls
- Large joints: Knee, ankle, elbow
- Iridocyclitis in up to 30%
- Arthritis of < 5 joints in first 6 months of disease
- Polyarticular rheumatoid factor (RF)-positive arthritis
- Arthritis of ≥ 5 joints in first 6 months of disease
- ≥ 2 positive tests for RF at least 3 months apart within first 6 months of disease
- More commonly adolescent girls
- Typical: Symmetric, small joints of hands
- Polyarticular RF-negative arthritis
- Arthritis of ≥ 5 joints in first 6 months of disease
- No characteristic pattern
- Any age throughout childhood
- Psoriatic arthritis (< 15%)
- Arthritis & psoriasis or arthritis & ≥ 2 of following: Dactylitis ("sausage digit"), nail pitting or onycholysis, psoriasis in 1st-degree relative
- Enthesitis-related arthritis (< 7%)
- Arthritis & enthesitis, or
- Arthritis or enthesitis with ≥ 2 of following
- Sacroiliac joint tenderness &/or inflammatory lumbosacral pain
- Positive HLA-B27 antigen test
- Onset of arthritis in male ≥ 6 years old
- Symptomatic anterior uveitis
- Presence of 1st-degree relative with ankylosing spondylitis, enthesitis-related arthritis, inflammatory bowel disease with sacroiliitis, Reiter syndrome, or acute anterior uveitis
- Enthesitis is most commonly at Achilles, plantar fascia
- Undifferentiated arthritis
- Does not fulfill criteria for any category or fulfills criteria for multiple categories
- Systemic arthritis (sJIA), previously known as Still disease: 10-20%
IMAGING
General Features
Radiographic Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Jana M et al: Role of imaging in childhood arthritis. J Clin Rheumatol. ePub, 2021
- Ntoulia A et al: Contrast-enhanced ultrasound for musculoskeletal indications in children. Pediatr Radiol. ePub, 2021
- Pracoń G et al: Conventional radiography and ultrasound imaging of rheumatic diseases affecting the pediatric population. Semin Musculoskelet Radiol. 25(1):68-81, 2021
- Schiettecatte E et al: MR imaging of rheumatic diseases affecting the pediatric population. Semin Musculoskelet Radiol. 25(1):82-93, 2021
- Malattia C et al: Current status of MR imaging of juvenile idiopathic arthritis. Best Pract Res Clin Rheumatol. 101629, 2020
- Malattia C et al: Tocilizumab may slow radiographic progression in patients with systemic or polyarticular-course juvenile idiopathic arthritis: post hoc radiographic analysis from two randomized controlled trials. Arthritis Res Ther. 22(1):211, 2020
- Lee JJY et al: Systemic juvenile idiopathic arthritis. Pediatr Clin North Am. 65(4):691-709, 2018
- Hemke R et al: Magnetic resonance imaging (MRI) of the knee as an outcome measure in juvenile idiopathic arthritis: an OMERACT reliability study on MRI scales. J Rheumatol. 44(8):1224-30, 2017
- Nguyen JC et al: US evaluation of juvenile idiopathic arthritis and osteoarticular infection. Radiographics. 37(4):1181-201, 2017
- Avenarius DF et al: Erosion or normal variant? 4-year MRI follow-up of the wrists in healthy children. Pediatr Radiol. 46(3):322-30, 2016
- Kumar S: Systemic juvenile idiopathic arthritis: diagnosis and management. Indian J Pediatr. 83(4):322-7, 2016
- Naredo E et al: One year in review: ultrasound in arthritis. Clin Exp Rheumatol. 34(1):1-10, 2016
- Nusman CM et al: Contrast-enhanced MRI of the knee in children unaffected by clinical arthritis compared to clinically active juvenile idiopathic arthritis patients. Eur Radiol. 26(4):1141-8, 2016
- Viswanathan V et al: Management of children with juvenile idiopathic arthritis. Indian J Pediatr. 83(1):63-70, 2016
- Carrasco R: Juvenile idiopathic arthritis overview and involvement of the temporomandibular joint: prevalence, systemic therapy. Oral Maxillofac Surg Clin North Am. 27(1):1-10, 2015
- Ording Muller LS et al: The joints in juvenile idiopathic arthritis. Insights Imaging. 6(3):275-84, 2015
- Hemke R et al: One-year followup study on clinical findings and changes in magnetic resonance imaging-based disease activity scores in juvenile idiopathic arthritis. J Rheumatol. 41(1):119-27, 2014
- Hemke R et al: Reliability and responsiveness of the Juvenile Arthritis MRI Scoring (JAMRIS) system for the knee. Eur Radiol. 23(4):1075-83, 2013
- Hemke R et al: The diagnostic accuracy of unenhanced MRI in the assessment of joint abnormalities in juvenile idiopathic arthritis. Eur Radiol. 23(7):1998-2004, 2013
- Sheybani EF et al: Imaging of juvenile idiopathic arthritis: a multimodality approach. Radiographics. 33(5):1253-73, 2013
- Beresford MW: Juvenile idiopathic arthritis: new insights into classification, measures of outcome, and pharmacotherapy. Paediatr Drugs. 13(3):161-73, 2011
- Prakken B et al: Juvenile idiopathic arthritis. Lancet. 377(9783):2138-49, 2011
- Beresford MW et al: New advances in the management of juvenile idiopathic arthritis--2: the era of biologicals. Arch Dis Child Educ Pract Ed. 94(5):151-6, 2009
- Vastert SJ et al: Systemic JIA: new developments in the understanding of the pathophysiology and therapy. Best Pract Res Clin Rheumatol. 23(5):655-64, 2009
- McGhee JL et al: Clinical utility of antinuclear antibody tests in children. BMC Pediatr. 4(1):13, 2004
- Flato B et al: Prognostic factors in juvenile rheumatoid arthritis: a case-control study revealing early predictors and outcome after 14.9 years. J Rheumatol. 30(2):386-93, 2003
- Ramanan AV et al: Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. J Rheumatol. 30(2):401-3, 2003
- Cassidy JT et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum. 29(2):274-81, 1986
