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Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management

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Abstract

Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. Recent data suggests that early cytokine blockage might abrogate chronic, destructive, therapy resistant arthritis phase, reflecting a potential “window of opportunity” in the care of children with sJIA.

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References

  1. Still GF. On a form of chronic joint disease in children. Med Chir Trans. 1897;80:47–60.9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Behrens EM, Beukelman T, Gallo L, et al. Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR). J Rheumatol. 2008;35:343–8.

    PubMed  Google Scholar 

  3. Russo RA, Katsicas MM. Patients with very early-onset systemic juvenile idiopathic arthritis exhibit more inflammatory features and a worse outcome. J Rheumatol. 2013;40:329–34.

    Article  CAS  PubMed  Google Scholar 

  4. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: Second revision, Edmonton 2001. J Rheumatol. 2004;31:390–2.

    PubMed  Google Scholar 

  5. Calabro JJ. Other extraarticular manifestations of juvenile rheumatoid arthritis. Arthritis Rheum. 1977;20:237–40.

    Article  CAS  PubMed  Google Scholar 

  6. Ishihara M, Nakamura S, Okada K, Yokota S, Ohno S. A case of juvenile rheumatoid arthritis with panuveitis. Nihon Ganka Gakkai Zasshi. 1992;96:109–13.

    CAS  PubMed  Google Scholar 

  7. Moore AT, Morin JD. Bilateral acquired inflammatory Brown’s syndrome. J Pediatr Ophthalmol Strabismus. 1985;22:26–30.

    CAS  PubMed  Google Scholar 

  8. Wang FM, Wertenbaker C, Behrens MM, Jacobs JC. Acquired Brown’s syndrome in children with juvenile rheumatoid arthritis. Ophthalmology. 1984;91:23–6.

    Article  CAS  PubMed  Google Scholar 

  9. Avcin T, Silverman ED, Forte V, Schneider R. Nasal septal perforation: a novel clinical manifestation of systemic juvenile idiopathic arthritis/adult onset Still’s disease. J Rheumatol. 2005;32:2429–31.

    PubMed  Google Scholar 

  10. Woo P. Systemic juvenile idiopathic arthritis: diagnosis, management, and outcome. Nat Clin Pract Rheumatol. 2006;2:28–34.

    Article  PubMed  Google Scholar 

  11. Schnitzer TJ, Ansell BM. Amyloidosis in juvenile chronic polyarthritis. Arthritis Rheum. 1977;20:245–52.

    CAS  PubMed  Google Scholar 

  12. Schneider R, Laxer RM. Systemic onset juvenile rheumatoid arthritis. Baillieres Clin Rheumatol. 1998;12:245–71.

    Article  CAS  PubMed  Google Scholar 

  13. Correll CK, Binstadt BA. Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis. Pediatr Res. 2014;75:176–83.

    Article  CAS  PubMed  Google Scholar 

  14. Mellins ED, Macaubas C, Grom AA. Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions. Nat Rev Rheumatol. 2011;7:416–26.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Donn R, Ellison S, Lamb R, Day T, Baildam E, Ramanan AV. Genetic loci contributing to hemophagocytic lymphohistiocytosis do not confer susceptibility to systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 2008;58:869–74.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child. 2001;85:421–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Bechtold S, Ripperger P, Dalla Pozza R, et al. Growth hormone increases final height in patients with juvenile idiopathic arthritis: data from a randomized controlled study. J Clin Endocrinol Metab. 2007;92:3013–8.

    Article  CAS  PubMed  Google Scholar 

  18. Spiegel LR, Schneider R, Lang BA, et al. Early predictors of poor functional outcome in systemic-onset juvenile rheumatoid arthritis: a multicenter cohort study. Arthritis Rheum. 2000;43:2402–9.

    Article  CAS  PubMed  Google Scholar 

  19. Martini A Systemic juvenile idiopathic arthritis. Autoimmun Rev. 2012;12:56–9.

    Article  CAS  PubMed  Google Scholar 

  20. Garcia-Carrasco M, Fuentes-Alexandro S, Escarcega RO, Rojas-Rodriguez J, Escobar LE. Efficacy of thalidomide in systemic onset juvenile rheumatoid arthritis. Joint Bone Spine. 2007;74:500–3.

    Article  PubMed  Google Scholar 

  21. Brinkman DM, de Kleer IM, ten Cate R, et al. Autologous stem cell transplantation in children with severe progressive systemic or polyarticular juvenile idiopathic arthritis: long-term follow-up of a prospective clinical trial. Arthritis Rheum. 2007;56:2410–21.

    Article  CAS  PubMed  Google Scholar 

  22. Verbsky JW, White AJ. Effective use of the recombinant interleukin 1 receptor antagonist anakinra in therapy resistant systemic onset juvenile rheumatoid arthritis. J Rheumatol. 2004;31:2071–5.

    PubMed  Google Scholar 

  23. Quartier P, Allantaz F, Cimaz R, et al. A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial). Ann Rheum Dis. 2011;70:747–54.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Nigrovic PA, Mannion M, Prince FH, et al. Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series. Arthritis Rheum. 2011;63:545–55.

    Article  CAS  PubMed  Google Scholar 

  25. Vastert SJ, de Jager W, Noordman BJ, et al. Effectiveness of first-line treatment with recombinant interleukin-1 receptor antagonist in steroid-naive patients with new-onset systemic juvenile idiopathic arthritis: results of a prospective cohort study. Arthritis Rheum. 2014;66:1034–43.

    Article  CAS  Google Scholar 

  26. Ruperto N, Brunner HI, Quartier P, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367:2396–406.

    Article  CAS  PubMed  Google Scholar 

  27. Lovell DJ, Giannini EH, Reiff AO, et al. Long-term safety and efficacy of rilonacept in patients with systemic juvenile idiopathic arthritis. Arthritis Rheum. 2013;65:2486–96.

    Article  CAS  PubMed  Google Scholar 

  28. Yokota S, Miyamae T, Imagawa T, et al. Therapeutic efficacy of humanized recombinant anti-interleukin-6 receptor antibody in children with systemic-onset juvenile idiopathic arthritis. Arthritis Rheum. 2005;52:818–25.

    Article  CAS  PubMed  Google Scholar 

  29. Yokota S, Imagawa T, Mori M, et al. Efficacy and safety of tocilizumab in patients with systemic-onset juvenile idiopathic arthritis: a randomised, double- blind, placebo-controlled, withdrawal phase III trial. Lancet. 2008;371:998–1006.

    Article  CAS  PubMed  Google Scholar 

  30. Yokota S, Imagawa T, Mori M, et al. Long-term treatment of systemic juvenile idiopathic arthritis with tocilizumab: results of an open label extension study in Japan. Ann Rheum Dis. 2013;72:627–8.

    Article  CAS  PubMed  Google Scholar 

  31. De Benedetti F, Brunner HI, Ruperto N, et al. Randomized trial of tocilizumab in systemic juvenileidiopathic arthritis. N Engl J Med 2012;367:2385–95.

  32. Russo RA, Katsicas MM. Clinical remission in patients with systemic juvenile idiopathic arthritis treated with anti-tumor necrosis factor agents. J Rheumatol. 2009;36:1078–82.

    Article  CAS  PubMed  Google Scholar 

  33. Nigrovic PA. Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheum. 2014;66:1405–13.

    Article  CAS  Google Scholar 

  34. Beukelman T, Patkar NM, Saag KG, 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;63:465–82.

    Article  Google Scholar 

  35. 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 Care Res (Hoboken). 2013;65:1551–63.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Christian Medical College, Vellore, Tamil Nadu, 632004, India

    Sathish Kumar

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  1. Sathish Kumar
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Correspondence to Sathish Kumar.

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Kumar, S. Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management. Indian J Pediatr 83, 322–327 (2016). https://doi.org/10.1007/s12098-016-2060-z

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  • DOI: https://doi.org/10.1007/s12098-016-2060-z

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