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Juvenile Idiopathic Arthritis
Christopher G. Anton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Juvenile idiopathic arthritis (JIA)
        • Synonyms

          • Juvenile rheumatoid arthritis (JRA), juvenile chronic arthritis
        • Definitions

          • Synovial inflammation of unknown cause
          • ≥ 6 weeks of joint symptoms
          • Begins < 16 years of age
          • Most widely used classification developed by International League of Associations for Rheumatology (ILAR)
            • Systemic arthritis (Still disease): 10-20%
              • Arthritis of ≥ 1 joint + daily spiking fever for ≥ 3 consecutive days
              • Accompanied by 1 or more 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%
            • 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 most commonly at Achilles, plantar fascia
            • Undifferentiated arthritis
              • Does not fulfill criteria for any category or fulfills criteria for multiple categories

        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

                                    Selected References

                                    1. 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
                                    2. Kumar S: Systemic Juvenile idiopathic arthritis: diagnosis and management. Indian J Pediatr. 83(4):322-7, 2016
                                    3. Naredo E et al: One year in review: ultrasound in arthritis. Clin Exp Rheumatol. 34(1):1-10, 2016
                                    4. 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
                                    5. Viswanathan V et al: Management of children with juvenile idiopathic arthritis. Indian J Pediatr. 83(1):63-70, 2016
                                    6. 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
                                    7. Ording Muller LS et al: The joints in juvenile idiopathic arthritis. Insights Imaging. 6(3):275-84, 2015
                                    8. 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
                                    9. 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
                                    10. 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
                                    11. Sheybani EF et al: Imaging of juvenile idiopathic arthritis: a multimodality approach. Radiographics. 33(5):1253-73, 2013
                                    12. Beresford MW: Juvenile idiopathic arthritis: new insights into classification, measures of outcome, and pharmacotherapy. Paediatr Drugs. 13(3):161-73, 2011
                                    13. 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
                                    14. 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
                                    15. McGhee JL et al: Clinical utility of antinuclear antibody tests in children. BMC Pediatr. 4(1):13, 2004
                                    16. 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
                                    17. 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
                                    18. Cassidy JT et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum. 29(2):274-81, 1986
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Juvenile idiopathic arthritis (JIA)
                                          • Synonyms

                                            • Juvenile rheumatoid arthritis (JRA), juvenile chronic arthritis
                                          • Definitions

                                            • Synovial inflammation of unknown cause
                                            • ≥ 6 weeks of joint symptoms
                                            • Begins < 16 years of age
                                            • Most widely used classification developed by International League of Associations for Rheumatology (ILAR)
                                              • Systemic arthritis (Still disease): 10-20%
                                                • Arthritis of ≥ 1 joint + daily spiking fever for ≥ 3 consecutive days
                                                • Accompanied by 1 or more 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%
                                              • 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 most commonly at Achilles, plantar fascia
                                              • Undifferentiated arthritis
                                                • Does not fulfill criteria for any category or fulfills criteria for multiple categories

                                          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

                                                                      Selected References

                                                                      1. 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
                                                                      2. Kumar S: Systemic Juvenile idiopathic arthritis: diagnosis and management. Indian J Pediatr. 83(4):322-7, 2016
                                                                      3. Naredo E et al: One year in review: ultrasound in arthritis. Clin Exp Rheumatol. 34(1):1-10, 2016
                                                                      4. 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
                                                                      5. Viswanathan V et al: Management of children with juvenile idiopathic arthritis. Indian J Pediatr. 83(1):63-70, 2016
                                                                      6. 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
                                                                      7. Ording Muller LS et al: The joints in juvenile idiopathic arthritis. Insights Imaging. 6(3):275-84, 2015
                                                                      8. 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
                                                                      9. 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
                                                                      10. 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
                                                                      11. Sheybani EF et al: Imaging of juvenile idiopathic arthritis: a multimodality approach. Radiographics. 33(5):1253-73, 2013
                                                                      12. Beresford MW: Juvenile idiopathic arthritis: new insights into classification, measures of outcome, and pharmacotherapy. Paediatr Drugs. 13(3):161-73, 2011
                                                                      13. 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
                                                                      14. 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
                                                                      15. McGhee JL et al: Clinical utility of antinuclear antibody tests in children. BMC Pediatr. 4(1):13, 2004
                                                                      16. 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
                                                                      17. 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
                                                                      18. Cassidy JT et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum. 29(2):274-81, 1986