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Juvenile Ankylosing Spondylitis

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Textbook of Clinical Pediatrics

Definition

The term juvenile-onset ankylosing spondylitis (AS) refers to a disease, which onset of symptoms occurs before the age of 16 years and its main features are familial aggregation, strong association with HLA-B27, as well as peripheral arthritis and enthesitis at onset and sacroiliitis and spondylitis throughout the course of the disease. Juvenile-onset AS is the counterpart of adult-onset AS, the prototype of the group of disease and syndromes known as spondyloarthritis (SpA). Either in children or adults, the SpA group includes undifferentiated forms, psoriatic arthritis (PsA), reactive arthritis (ReA), ulcerative colitis, and Chron’s disease.

Diagnosis

Juvenile-onset AS is diagnosed according to the modified New York criteria for AS (Table 155.1 ). The fulfillment of such criteria requires clinical and radiographic evidence of spinal and sacroiliac joint involvement, particularly structural radiographic changes of the sacroiliac joints. (Fig. 155.1 ). Since AS in children...

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References

  • Ansell BM, Bywaters EG (1962) Diagnosis of “probable” Still's disease and its outcome. Ann Rheum Dis 21:253–262

    Article  PubMed  CAS  Google Scholar 

  • Bollow M, Biedermann T, Kannenberg J et al (1998) Use of dynamic magnetic resonance imaging to detect sacroiliitis in HLA-B27 positive and negative children with juvenile arthritides. J Rheumatol 25:556–564

    PubMed  CAS  Google Scholar 

  • Bowness P (2002) HLA B27 in health and disease: a double-edged sword? Rheumatology (Oxford) 41:857–868

    Article  CAS  Google Scholar 

  • Bowyer S, Roettcher P et al (1996) Pediatric Rheumatology Database Research Group. Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. J Rheumatol 23:1968–1974

    PubMed  CAS  Google Scholar 

  • Brown MA (2008) Breakthroughs in genetic studies of ankylosing spondylitis. Rheumatology 47:132–137

    Article  PubMed  CAS  Google Scholar 

  • Burgos Vargas R, Casasola-Vargas JC, Gutíerrez Suárez R et al (2008) An open, observational, extension study of a three month randomized placebo controlled trial to assess the long-term efficacy and safety of infliximab in Juvenile-onset Spondyloarthritis. Arthritis Rheum 9(Suppl):1103

    Google Scholar 

  • Burgos-Vargas R (2002) The juvenile-onset spondyloarthritides. Rheum Dis Clin North Am 28:531–560

    Article  PubMed  Google Scholar 

  • Burgos-Vargas R (2006) The juvenile-onset spondyloarthritides. In: Weisman MH, van der Heijde D, Reveille JD (eds) Ankylosing spondylitis and the spondyloarthropathies. Mosby, Philadelphia, pp 94–106

    Chapter  Google Scholar 

  • Burgos-Vargas R (2009) A case of childhood-onset ankylosing spondylitis: diagnosis and treatment. Nat Clin Pract Rheumatol 5:52–57

    Article  PubMed  Google Scholar 

  • Burgos-Vargas R, Clark P (1989) Axial involvement in the seronegative enthesopathy and arthropathy syndrome and its progression to ankylosing spondylitis. J Rheumatol 16:192–197

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Granados-Arriola J (1990) Ankylosing spondylitis and related diseases in the Mexican Mestizo. In: Khan MA (ed) Ankylosing spondylitis and related spondyloarthropathies. Spine: state of the art reviews, vol 4. Hanley & Belfus, Philadelphia, pp 655–678

    Google Scholar 

  • Burgos-Vargas R, Vázquez-Mellado J (1995) The early clinical recognition of juvenile-onset ankylosing spondylitis and its differentiation from juvenile rheumatoid arthritis. Arthritis Rheum 38:835–844

    Article  PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Lardizabal-Sanabria J, Katona G (1985) Anterior spinal flexion in healthy Mexican children. J Rheumatol 12:123–125

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Howard A, Ansell BM (1986) Antibodies to peptidoglycan in juvenile onset ankylosing spondylitis and pauciarticular onset juvenile arthritis associated with chronic iridocyclitis. J Rheumatol 13:760–765

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Naranjo A, Castillo J et al (1989) Ankylosing spondylitis in the Mexican Mestizo: patterns of disease according to age at onset. J Rheumatol 16:186–191

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Castelazo-Duarte G, Orozco JA et al (1993) Chest expansion in healthy adolescents and in patients with juvenile ankylosing spondylitis or the seronegative enthesopathy and arthropathy syndrome. J Rheumatol 20:1957–1969

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Vázquez-Mellado J, Cassis N et al (1996) Genuine ankylosing spondylitis in children: a case control study of patients with definite disease according to current adult-onset criteria shortly after onset. J Rheumatol 23:2140–2147

    PubMed  CAS  Google Scholar 

  • Burgos-Vargas R, Vázquez-Mellado J, Pacheco-Tena C et al (2002) A 26 week randomised, double blind, placebo controlled exploratory study of sulfasalazine in juvenile onset spondyloarthropathies. Ann Rheum Dis 61:941–942

    Article  PubMed  CAS  Google Scholar 

  • Cabral DA, Oen KG, Petty RE (1992) SEA syndrome revisited: a longterm followup of children with a syndrome of seronegative enthesopathy and arthropathy. J Rheumatol 19:1282–1285

    PubMed  CAS  Google Scholar 

  • Calin A, Elswood S (1988) The natural history of juvenile onset ankylosing spondylitis: 24 year retrospective case control study. Br J Rheumatol 27:91–93

    Article  PubMed  CAS  Google Scholar 

  • Carter KW, Pluzhnikov A, Timms AE et al (2007) Combined analysis of three whole genome linkage scans for ankylosing spondylitis. Rheumatology 46:763–771

    Article  PubMed  CAS  Google Scholar 

  • Colbert RA (2000) HLA-B27 misfolding: a solution to the spondyloarthropathy conundrum? Mol Med Today 6:224–230

    Article  PubMed  CAS  Google Scholar 

  • Consortium WTCC (2007) Genomewide association study of 14,000 cases of seven common diseases and 3000 controls. Nature 447:661–683

    Article  Google Scholar 

  • Dangoria NS, DeLay ML, Kingsbury DJ et al (2002) HLA-B27 misfolding is associated with aberrant intermolecular disulfide bond formation (dimerization) in the endoplasmic reticulum. J Biol Chem 28:23459–23468

    Article  Google Scholar 

  • Denardo BA, Tucker LB, Miller LC et al (1994) Demography of a regional pediatric rheumatology patient population. J Rheumatol 21:1553–1561

    PubMed  CAS  Google Scholar 

  • Dougados M, van der Linden S, Juhlin R et al (1991) The European Spondyloarthropathy Study Group preliminary criteria for the classification of spondyloarthropathy. Arthritis Rheum 34:1218–1227

    Article  PubMed  CAS  Google Scholar 

  • Duarte-Salazar C, Guzmán-Vázquez S, Soto-Molina H et al (2007) Disability impact on quality of life in Mexican adults with juvenile idiopathic arthritis and juvenile ankylosing spondylitis. Clin Exp Rheumatol 25:922–927

    PubMed  CAS  Google Scholar 

  • Edmonds J, Morris RI, Metzger AL, Bluestone R, Terasaki PI, Ansell B, Bywaters EG (1974) Follow-up study of juvenile chronic polyarthritis with particular reference to histocompatibility antigen W. 27. Ann Rheum Dis 33:289–292

    Article  PubMed  CAS  Google Scholar 

  • Edstrom G, Thune S, Wittbom-Cigen G (1960) Juvenile ankylosing spondylitis. Acta Rheumatol Scand 6:161–173

    Article  PubMed  CAS  Google Scholar 

  • Fiorillo MT, Maragno M, Butler R et al (2000) CD8(+) T-cell autoreactivity to an HLA-B27-restricted self-epitope correlates with ankylosing spondylitis. J Clin Invest 106:47–53

    Article  PubMed  CAS  Google Scholar 

  • Flatø B, Hoffmann-Vold AM, Reiff A et al (2006) Long-term outcome and prognostic factors in enthesitis-related arthritis: a case-control study. Arthritis Rheum 54:3573–3582

    Article  PubMed  Google Scholar 

  • Flato B, Smerdel A, Johnston V et al (2002) The influence of patient characteristics, disease variables, and HLA alleles on the development of radiographically evident sacroiliitis in juvenile idiopathic arthritis. Arthritis Rheum 46:986–994

    Article  PubMed  Google Scholar 

  • Gensler LS, Ward MM, Reveille JD et al (2008) Clinical, radiographic and functional differences between juvenile-onset and adult-onset ankylosing spondylitis: results from the PSOAS cohort. Ann Rheum Dis 67:233–237

    Article  PubMed  CAS  Google Scholar 

  • Grom AA, Murray KJ, Luyrink L et al (1996) Patterns of expression of tumor necrosis factor α, tumor necrosis factor β, and their receptors in synovia of patients with juvenile rheumatoid arthritis and juvenile spondylarthropathy. Arthritis Rheum 39:1703–1710

    Article  PubMed  CAS  Google Scholar 

  • Hall MA, Burgos-Vargas R, Ansell BM (1987) Sacroiliitis in juvenile chronic arthritis: a 10-year follow-up. Clin Exp Rheumatol 5(Suppl):65–67

    Google Scholar 

  • Henrickson M, Reiff A (2004) Prolonged efficacy of etanercept in refractory enthesitis-related arthritis. J Rheumatol 31:2055–2061

    PubMed  CAS  Google Scholar 

  • Jacobs JC, Johnston AD, Berdon WE (1982) HLA-B27 associated spondyloarthritis and enthesopathy in childhood: clinical, pathologic and radiographic observations in 58 patients. J Pediatr 100:521–528

    Article  PubMed  CAS  Google Scholar 

  • Jiménez-Balderas FJ, García-Rubi D, Pérez-Hinojosa S et al (2001) Two-dimensional echo Doppler findings in juvenile and adult onset ankylosing spondylitis with long-term disease. Angiology 52:543–548

    Article  PubMed  Google Scholar 

  • Kruithof E, Van den Bossche V, De Rycke L et al (2006) Distinct synovial immunopathologic characteristics of juvenile-onset spondylarthritis and other forms of juvenile idiopathic arthritis. Arthritis Rheum 54:2594–2604

    Article  PubMed  CAS  Google Scholar 

  • Ladd JR, Cassidy JT, Martel W (1971) Juvenile ankylosing spondylitis. Arthritis Rheum 14:579–590

    Article  PubMed  CAS  Google Scholar 

  • Lau CS, Burgos-Vargas R, Louthrenoo W et al (1998) Features of spondyloarthropathies around the world. Rheum Dis Clin North Am 24:753–770

    Article  PubMed  CAS  Google Scholar 

  • López-Larrea C, Gonzalez-Roces S, Peña M et al (1995) Characterization of B27 haplotypes by oligotyping and genomic sequencing in the Mexican Mestizo population with ankylosing spondylitis: juvenile and adult onset. Hum Immunol 43:174–180

    Article  PubMed  Google Scholar 

  • Maksymowych P, Luong M, Wong C et al (1997) The LMP2 polymorphism is associated with susceptibility to acute anterior uveitis in HLA-B27 positive juvenile and adult Mexican individuals with AS. Ann Rheum Dis 56:807–814

    Google Scholar 

  • Malleson PN, Fung MY, Rosenberg AM et al (1996) The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry. J Rheumatol 23:1981–1987

    PubMed  CAS  Google Scholar 

  • Marks S, Bennett M, Calin A (1982) The natural history of juvenile ankylosing spondylitis: a case control study of juvenile and adult onset disease. J Rheumatol 9:739–741

    PubMed  CAS  Google Scholar 

  • McGonagle D, Marzo-Ortega H, O'Connor P et al (2002) Histological assessment of the early enthesitis lesion in spondyloarthropathy. Ann Rheum Dis 61:534–537

    Article  PubMed  CAS  Google Scholar 

  • Minden K, Kiessling U, Listing J et al (2000) Prognosis of patients with juvenile chronic arthritis and juvenile spondyloarthropathy. J Rheumatol 27:2256–2263

    PubMed  CAS  Google Scholar 

  • Minden K, Niewerth M, Listing J, Biedermann T et al (2002) Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 46:2392–2401

    Article  PubMed  Google Scholar 

  • Murray KJ, Grom AA, Thompson SD et al (1998) Contrasting cytokine profiles in the synovium of different forms of juvenile rheumatoid arthritis and juvenile spondyloarthropathy: prominence of interleukin 4 in restricted disease. J Rheumatol 25:1388–1398

    PubMed  CAS  Google Scholar 

  • Pacheco-Tena C, Alvarado de la Barrera C, López-Vidal Y et al (2001) Bacterial DNA in synovial fluid cells of patients with juvenile onset spondyloarthropathies. Rheumatology 40:920–927

    Article  PubMed  CAS  Google Scholar 

  • Petty RE, Southwood TR, Manners P et al (2004) International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton (2001). J Rheumatol 31:390–392

    PubMed  Google Scholar 

  • Ploski R, Flato B, Vinje O et al (1995) Association to HLA-DRB108, HLA-DPB10301 and homozygosity for an HLA-linked proteasome gene in juvenile ankylosing spondylitis. Hum Immunol 44:88–96

    Article  PubMed  CAS  Google Scholar 

  • Ramos M, Lopez de Castro JA (2002) HLA-B27 and the pathogenesis of spondyloarthritis. Tissue Antigens 60:191–205

    Article  PubMed  CAS  Google Scholar 

  • Riley MJ, Ansell BM, Bywaters EG (1971) Radiological manifestations of ankylosing spondylitis according to age at onset. Ann Rheum Dis 30:138–148

    Article  PubMed  CAS  Google Scholar 

  • Rosenberg AM (2005) Longitudinal analysis of a pediatric rheumatology clinic population. Rheumatology 32:1992–2001

    Google Scholar 

  • Rosenberg AM, Petty RE (1982) A syndrome of seronegative enthesopathy and arthropathy in children. Arthritis Rheum 25:1041–1047

    Article  PubMed  CAS  Google Scholar 

  • Rudwaleit M, Metter A, Listing J et al (2006) Inflammatory back pain in ankylosing spondylitis: a reassessment of the clinical history for application as classification and diagnostic criteria. Arthritis Rheum 54:569–578

    Article  PubMed  Google Scholar 

  • Schaller JG, Bitnum S, Wedgwood RJ (1969) Ankylosing spondylitis with childhood onset. J Pediatr 74:505–516

    Article  PubMed  CAS  Google Scholar 

  • Scott SG (1942) A monograph on adolescent spondylitis or ankylosing spondylitis, the early diagnosis and its treatment by wide-field x-ray irradiation. Oxford University Press, London

    Google Scholar 

  • Sheerin KA, Giannini EH, Brewer EJ et al (1988) HLA-B27-associated arthropathy in childhood: long-term clinical and diagnostic outcome. Arthritis Rheum 31:1165–1170

    Article  PubMed  CAS  Google Scholar 

  • Stamato T, Laxer RM, de Freitas C et al (1995) Prevalence of cardiac manifestations of juvenile ankylosing spondylitis. Am J Cardiol 75:744–746

    Article  PubMed  CAS  Google Scholar 

  • Stone M, Warren RW, Bruckel J (2005) Juvenile-onset ankylosing spondylitis is associated with worse functional outcomes than adult-onset ankylosing spondylitis. Arthritis Rheum 53:445–451

    Article  PubMed  Google Scholar 

  • Sulpice M, Deslandre CJ, Quartier P (2009) Efficacy and safety of TNF-alpha antagonist therapy in patients with juvenile spondyloarthropathies. Joint Bone Spine 76:24–27

    Article  PubMed  CAS  Google Scholar 

  • Symmons DPM, Jones M, Osborne J et al (1996) Pediatric rheumatology in the United Kingdom: data from the British Pediatric Rheumatology Group National Diagnostic Register. J Rheumatol 23:1975–1980

    PubMed  CAS  Google Scholar 

  • Tse SM, Burgos-Vargas R, Laxer RM (2005) Anti-tumor necrosis factor alpha blockade in the treatment of juvenile spondylarthropathy. Arthritis Rheum 52:2103–2108

    Article  PubMed  CAS  Google Scholar 

  • Uchanska-Ziegler B, Ziegler A (2003) Ankylosing spondylitis: a beta2m-deposition disease? Trends Immunol 24:73–76

    Article  PubMed  CAS  Google Scholar 

  • Van der Linden S, Valkenburg HA, Cats A (1984) Evaluation of diagnostic criteria for ankylosing spondylitis. A proposal for modification of the New York Criteria. Arthritis Rheum 27:361–368

    Article  PubMed  Google Scholar 

  • Zou J, Appel H, Rudwaleit M et al (2005) Analysis of the CD8+ T cell response to the G1 domain of aggrecan in ankylosing spondylitis. Ann Rheum Dis 64:722–729

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Rubén Burgos-Vargas M.D. .

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Burgos-Vargas, R., Gutiérrez-Suárez, R. (2012). Juvenile Ankylosing Spondylitis. In: Elzouki, A.Y., Harfi, H.A., Nazer, H.M., Stapleton, F.B., Oh, W., Whitley, R.J. (eds) Textbook of Clinical Pediatrics. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02202-9_155

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