Skip to main content

Advertisement

Log in

Juvenile Spondyloarthropathies

  • Pediatric Rheumatology (M Stoll, Guest Section Editor)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Juvenile spondyloarthropathies represent a clinical entity separate from the adult disease. Initial clinical signs of juvenile spondyloarthropathies often include lower extremity arthritis and enthesopathy, without axial involvement at the disease onset. Asymmetrical oligoarthritis of lower extremities is typically seen in this type of arthritis. Enthesopathy, which is the hallmark of the disease, is most commonly seen in the Achilles tendon, being manifested by heel pain. Anterior uveitis and HLA-B27 positivity are seen in a proportion of cases. Sacroiliitis is generally asymptomatic in the pediatric population. Ineffective treatment of childhood disease results in disease progression to typical adult form of ankylosing spondylitis. Therefore, early diagnosis and classification remains one of the most relevant questions in pediatric rheumatology. It should be kept in mind that the disease could be misdiagnosed as FMF or Behçet’s syndrome in countries with a high incidence of those conditions. This review revises available classification criteria, clinical manifestations and therapeutic options for patients with juvenile spondyloarthropathies.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Tse SML, Laxer RM. New advances in juvenile spondyloarthritis. Nat Rev Rheumatol. 2012;8:269–79. An up-to date review of advances in the classification, diagnosis and management strategies for juvenile spondyloarthritis.

    Article  CAS  PubMed  Google Scholar 

  2. Kasapcopur O, Ayaz AN, Apelyan M, Ozdogan H, Arisoy N. Juvenile-onset Spondyloarthropathies. Current Options in Pediatric Rheumatology. A guide for general practitioners and pediatricians. New York: Nova Science Publisher; 2006.

    Google Scholar 

  3. Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, 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 

  4. Sieper J, Rudwaleit M, Baraliakos X, Brandt J, Braun J, Burgos-Vargas R, et al. The Assessment of SpondyloArthritis international Society (ASAS) handbook: a guide to assess spondyloarthritis. Ann Rheum Dis. 2009;68:1–44. Fundamental manuscript on the most relevant aspects for the assessment of spondyloarthritis.

    Google Scholar 

  5. Kasapcopur O, Demirli N, Ozdogan H, Apelyan M, Caliskan S, Sever L, et al. Evaluation of classification criteria for juvenile-onset spondyloarthropathies. Rheumatol Int. 2004;25:414–8.

    Article  PubMed  Google Scholar 

  6. Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic diseses: results from the Canadian pediatric rheumatology association disease registry. J Rheumatol. 1996;23:1981–7.

    CAS  PubMed  Google Scholar 

  7. Oen K, Fast M, Postl BL. Epidemiology of juvenile rheumatoid arthritis in Manitoba, Canada: cycles in incidence. J Rheumatol. 1995;22:745–50.

    CAS  PubMed  Google Scholar 

  8. Weiss PF, Beukelman T, Schanberg LE, Kimura Y, Colbert RA. CARRA Registry Investigators Enthesitis-related arthritis is associated with higher pain intensity and poorer health status in comparison with other categories of juvenile idiopathic arthritis: the Childhood Arthritis and Rheumatology Research Alliance Registry. J Rheumatol. 2012;39:2341–51.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Beukelman T, Ringold S, Davis TE, DeWitt EM, Pelajo CF, Weiss PF, et al. Disease-modifying antirheumatic drug use in the treatment of juvenile idiopathic arthritis: a cross-sectional analysis of the CARRA Registry. J Rheumatol. 2012;39:1867–74.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Kasapçopur O, Yologlu N, Ozyazgan Y, Ercan G, Caliskan S, Sever L, et al. Uveitis and anti nuclear antibody positivity in children with juvenile idiopathic arthritis. Indian Pediatr. 2004;41:1035–9.

    PubMed  Google Scholar 

  11. Ozdogan H, Kasapcopur O, Dede H, Arisoy N, Beceren T, Yurdakul S, et al. Juvenile chronic arthritis in a Turkish population. Clin Exp Rheumatol. 1991;9:431–5.

    CAS  PubMed  Google Scholar 

  12. Demirkaya E, Ozen S, Bilginer Y, Ayaz N, Makay B, Unsal E, et al. The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association. Clin Exp Rheumatol. 2011;29:111–6. A relevant cross-sectional study of demographics, clinical and laboratory features and subtype distribution of juvenile idiopathic arthritis in an eastern Mediterranean country, based on a multicenter registry.

    PubMed  Google Scholar 

  13. Reveille JD. Genetics of spondyloarthritis beyond the MHC. Nat Rev Rheumatol. 2012;8:296–304.

    Article  CAS  PubMed  Google Scholar 

  14. Aggarwal A, Misra DP. Enthesitis-related arthritis. Clin Rheumatol. 2015;34:1839–46. Brief description of the pathogenesis, clinical features and treatment options for enthesitis-related arthritis.

    Article  PubMed  Google Scholar 

  15. Jacques P, Elewaut D. Gut inflammation in SpA: flagellin joins the dance? Nat Rev Rheumatol. 2014;10:5–6.

    Article  PubMed  Google Scholar 

  16. Sezen M, Barut K, Açıkel C, Kasapcopur O. The new proposal classification criteria for juvenile spondyloarthropathies [abstract]. Arthritis Rheumatol. 2014;66:S1004(2301) (suppl 10).

  17. Dougados M, Baeten D. Spondyloarthritis. Lancet. 2011;377:2127–37.

    Article  PubMed  Google Scholar 

  18. Rudwaleit M, van der Heijde D, Landewe R, et al. The Assessment of SpondyloArthritis International Society classification criteria for peripheral spondyloarthritis and for spondyloarthritis in general. Ann Rheum Dis. 2011;70:25–31. Comparison of The Assessment of Spondylo Arthritis International Societ (ASAS) classification criteria with the European Spondylarthropathy Study Group (ESSG) and Amor criteria which shows that the new ASAS classification criteria for peripheral SpA performed well in patients presenting with peripheral arthritis, enthesitis and/or dactylitis.

    Article  CAS  PubMed  Google Scholar 

  19. Rudwaleit M, van der Heijde D, Landewe R, Listing J, Akkoc N, Brandt J, et al. The development of Assessment of SpondyloArthritis international Society classification criteria for axial spondyloarthritis (part II): validation and final selection. Ann Rheum Dis. 2009;68:777–83. ASAS report that validates and refines two sets of criteria for the classification and diagnosis of axial spondyloarthritis.

    Article  CAS  PubMed  Google Scholar 

  20. Burgos-Vargas R. The assessment of the spondyloarthritis international society concept and criteria for the classification of axial spondyloarthritis and peripheral spondyloarthritis: a critical appraisal for the pediatric rheumatologist. Pediatr Rheumatol. 2012;10:14. Excellent assessment of the spondyloarthritis international society concept and criteria for the classification of axial spondyloarthritis and peripheral spondyloarthritis with a special emphasis on the pediatric population.

    Article  Google Scholar 

  21. Duartea AP, Marquesa CDL, Bortoluzzob AB, Goncalvesc CR, da Silvad JA B, Ximenese AC, et al. Epidemiologic profile of juvenile-onset compared to adult-onset spondyloarthritis in a large Brazilian cohort. Rev Bras Reumatol. 2014;54:424–30. Prospective, multicenter analysis of a large Brazilian cohort with 1424 patients with the diagnosis of SpA revealing the main differences among juvenile-onset and adult-onset spondyloarthritis patients.

    Article  Google Scholar 

  22. Stone M, Warren RW, Bruckel J, Cooper D, Cortinovis D, Inman RD, et al. Juvenile-onset ankylosing spondylitis is associated with worse functional outcomes than adult-onset ankylosing spondylitis. Arthritis Rheum. 2005;53:445–51.

    Article  PubMed  Google Scholar 

  23. Alvarez-Madrid C, Merino R, Inocencio JD, Garcia-Consuegra J. Tarsitis as an initial manifestation of juvenile spondyloarthropathy. Clin Exp Rheumatol. 2009;27:691–4.

    CAS  PubMed  Google Scholar 

  24. Barut K, Sezen M, Sahin S, Adrovic A, Ugurlu S, Ozdogan H, et al. Juvenile Spondyloarthropathies: a Single Center Experience [abstract]. Ann Rheum Dis. 2015;74 suppl 2:390.

    Article  Google Scholar 

  25. van der Heijde D, Lie E, Kvien TK, Sieper J, Van den Bosch F, Listing J, et al. ASDAS, a highly discriminatory ASAS-endorsed disease activity score in patients with ankylosing spondylitis. Ann Rheum Dis. 2009;68:1811–8.

    Article  PubMed  Google Scholar 

  26. Weiss PF, Colbert RA, Xiao R, Feudtner C, Beukelman T, Dewitt EM, et al. Development and retrospective validation of the juvenile spondyloarthritis disease activity index. Arthritis Care Res. 2014;66:1775–82.

    Article  Google Scholar 

  27. Saurenmann RK, Rose JB, Tyrrell P, Feldman BM, Laxer RM, Schneider R, et al. Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort: ethnicity as a risk factor. Arthritis Rheum. 2007;56:1974–84.

    Article  CAS  PubMed  Google Scholar 

  28. Henderson LA, Zurakowski D, Angeles-Han ST, Lasky A, Rabinovich CE, Lo MS, et al. Medication use in juvenile uveitis patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry. Pediatr Rheumatol Online J. 2016;14:9.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Kahn P. Juvenile idiopathic arthritis. An update for the clinican. Bull NYU Hosp Jt Dis. 2012;70:152–66.

    PubMed  Google Scholar 

  30. Burgos-Vargas R, Pacheco-Tena C, Vazquez-Mellado J. A short-term follow-up of enthesitis and arthritis in the active phase of juvenile onset spondyloarthropathies. Clin Exp Rheumatol. 2002;20:727–31.

    CAS  PubMed  Google Scholar 

  31. Berntson L, Damgard M, Andersson-Gare B, Herlin T, Nielsen S, Nordal E, et al. HLA-B27 predicts a more extended disease with increasing age at onset in boys with juvenile idiopathic arthritis. J Rheumatol. 2008;35:2055–61.

    CAS  PubMed  Google Scholar 

  32. Saraux A, Benichou J, Guillevin L, Idbrik L, Job-Deslandre C, Sibilia J, et al. Which patients with rheumatoid arthritis, spondyloarthritis, or juvenile idiopathic arthritis receive TNF-α antagonists in France?The CORPUS cohort study. Clin Exp Rheumatol. 2015;33:602–10.

    PubMed  Google Scholar 

  33. Helliwell PS. Relationship of psoriatic arthritis with the other spondyloarthropathies. Curr Opin Rheumatol. 2004;16:344–9.

    Article  PubMed  Google Scholar 

  34. Ravelli A, Consolaro A, Schiappapietra B, Martini A. The conundrum of juvenile psoriatic arthritis. Clin Exp Rheumatol. 2015;33:40–3. A worthy manuscript which debates discordances in the definition and classification of juvenile psoriatic arthritis.

    Google Scholar 

  35. Taylor W, Gladman D, Helliwell P, Marchesoni A, Mease P, Mielants H, et al. Classification criteria for psoriatic arthritis: development of new criteria from a large international study. Arthritis Rheum. 2006;54:2665–73.

    Article  PubMed  Google Scholar 

  36. Vendhan K, Sen D, Fisher C, Ioannou Y, Hall-Cragg MA. Inflammatory changes of the lumbar spine in children and adolescents with enthesitis-related arthritis: magnetic resonance imaging findings. Arthritis Care Res. 2014;66:40–6. A considerable description of magnetic resonance imaging of lumbar spine abnormalities in a cohort of patients with enthesitis-related arthritis compared to a control group of adolescents with mechanical back pain.

    Article  Google Scholar 

  37. Yilmaz MH, Ozbayrak M, Kasapcopur O, Kurugoglu S, Kanberoglu K. Pelvic MRI findings of juvenile-onset ankylosing spondylitis. Clin Rheumatol. 2010;29:1007–13.

    Article  PubMed  Google Scholar 

  38. Weiss PF, Xiao R, Biko DM, Johnson AM, Chauvin NA. Detection of inflammatory sacroiliitis in children with magnetic resonance imaging: is gadolinium contrast enhancement necessary? Arthritis Rheumatol. 2015;67:2250–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Pagnini I, Savelli S, Matucci-Cerinic M, Fonda C, Cimaz R, Simonini G. Early predictors of juvenile sacroiliitis in enthesitis-related arthritis. J Rheumatol. 2010;37:2395–401.

    Article  PubMed  Google Scholar 

  40. 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;63:465–82.

    Article  Google Scholar 

  41. Paramarta JE, Baeten D. Spondyloarthritis: from unifying concepts to improved treatment. Rheumatology (Oxford). 2014;53:1547–59.

    Article  Google Scholar 

  42. Horneff G, Fitter S, Foeldvari I, Minden K, Kuemmerle-Deschner J, Tzaribacev N, et al. Double-blind, placebocontrolled randomized trial with adalimumab for treatment of juvenile onset ankylosing spondylitis (JoAS): significant short term improvement. Arthritis Res Ther. 2012;24:R230.

    Article  Google Scholar 

  43. Haibel H, Rudwaleit M, Listing J, Heldmann F, Wong RL, Kupper H, et al. Efficacy of adalimumab in the treatment of axial spondylarthritis without radiographically defined sacroiliitis: results of a twelve-week randomized, double-blind, placebo-controlled trial followed by an open-label extension up to week fifty-two. Arthritis Rheum. 2008;58:1981–91.

    Article  CAS  PubMed  Google Scholar 

  44. Sieper J, Lenaerts J, Wollenhaupt J, Rudwaleit M, Mazurov VI, Myasoutova L, et al. Efficacy and safety of infliximab plus naproxen versus naproxen alone in patients with early, active axial spondyloarthritis: results from the double-blind, placebo-controlled INFAST study, Part 1. Ann Rheum Dis. 2014;73:101–7.

    Article  CAS  PubMed  Google Scholar 

  45. Rudwaleit M, Schwarzlose S, Hilgert ES, Listing J, Braun J, Sieper J. MRI in predicting a major clinical response to anti-tumour necrosis factor treatment in ankylosing spondylitis. Ann Rheum Dis. 2008;67:1276–81.

    Article  CAS  PubMed  Google Scholar 

  46. Canoui Poitrine F, Poulain C, Molto A, Le T, Lafon C, Farrenq V, et al. Early tumor necrosis factor-antagonist therapy in everyday practice for inflammatory back pain suggesting axial spondyloarthritis: results from a prospective multicenter French cohort. Arthritis Care Res (Hoboken). 2014;66:1395–402. Data from a French prospective observational study that underlines the importance of early diagnosis and treatment of axial spondyloarthritis in order to achieve the best improvement of patient symptoms. Furthermore, it defines factors associated with early anti-TNF alpha initiation.

    Article  CAS  Google Scholar 

  47. Baeten D, Sieper J, Braun J, Sieper J, Emery P, van der Heijde D. Anti-interleukin-17A monoclonal antibody secukinumab in treatment of ankylosing spondylitis: a randomised, double-blind, placebo-controlled trial. Lancet. 2013;382:1705–13.

    Article  CAS  PubMed  Google Scholar 

  48. McInnes IB, Sieper J, Braun J, Emery P, van der Heijde D, Isaacs JD. Efficacy and safety of secukinumab, a fully human anti-interleukin-17A monoclonal antibody, in patients with moderate-tosevere psoriatic arthritis: a 24-week, randomised, double-blind, placebo-controlled, phase II proof-ofconcept trial. Ann Rheum Dis. 2014;73:349–56.

    Article  CAS  PubMed  Google Scholar 

  49. Gottlieb A, Menter A, Mendelsohn A, Shen YK, Li S, Guzzo C, et al. Ustekinumab, a human interleukin 12/23 monoclonal antibody, for psoriatic arthritis: randomised, doubleblind, placebo controlled, crossover trial. Lancet. 2009;373:633–40.

    Article  CAS  PubMed  Google Scholar 

  50. McInnes IB, Kavanaugh A, Gottlieb AB, Puig L, Rahman P, Ritchlin C, et al. Efficacy and safety of ustekinumab in patients with active psoriatic arthritis: 1 year results of the phase 3, multicentre, double-blind, placebo-controlled PSUMMIT 1 trial. Lancet. 2013;31:780–9.

    Article  Google Scholar 

  51. Hugle B, Burgos-Vargas R, Inman RD, O’Shea F, Laxer RM, Stimec J, et al. Long-term outcome of anti-tumour necrosis factor alpha blockade in the treatment of juvenile spondyloarthritis. Clin Exp Rheumatol. 2014;32:424–31. An observational study of 16 patients with JSpA from 3 centers that describes long-term efficacy of anti-TNF treatment in JSpA refractory to standard therapy.

    CAS  PubMed  Google Scholar 

  52. Jadon D, Shaddick G, Jobling A, Ramanan AV, Sengupta R. Clinical outcomes and progression to orthopedic surgery in juvenile versus adult onset ankylosing spondylitis. Arthritis Care Res (Hoboken). 2015;67:651–7.

    Article  Google Scholar 

  53. Prince FH, Geerdink LM, Borsboom GJ, Twilt M, van Rossum MA, Hoppenreijs EP, et al. Major improvements in health-related quality of life during the use of etanercept in patients with previously refractory juvenile idiopathic arthritis. Ann Rheum Dis. 2010;69:138–42.

    Article  CAS  PubMed  Google Scholar 

  54. Gerloni V, Pontikaki I, Gattinara M, Fantini F. Focus on adverse events of tumour necrosis factor alpha blockade in juvenile idiopathic arthritis in an open monocentric long-term prospective study of 163 patients. Ann Rheum Dis. 2008;67:1145–52.

    Article  CAS  PubMed  Google Scholar 

  55. Conway R, O'shea FD. Juvenile versus adult-onset ankylosing spondylitis: are we comparing apples and oranges? J Rheumatol. 2012;39:887–9.

    Article  PubMed  Google Scholar 

  56. Tse SM, Burgos-Vargas R, Laxer RM. Anti-tumor necrosis factor alpha blockade in the treatment of juvenile spondylarthropathy. Arthritis Rheum. 2005;52:2103–8.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ozgur Kasapcopur.

Ethics declarations

Conflicts of Interest

AA, KB, SS, and OK declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Pediatric Rheumatology

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Adrovic, A., Barut, K., Sahin, S. et al. Juvenile Spondyloarthropathies. Curr Rheumatol Rep 18, 55 (2016). https://doi.org/10.1007/s11926-016-0603-y

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11926-016-0603-y

Keywords

Navigation