Rheumaderm pp 27-33 | Cite as

Prognosis in Juvenile Arthritis

  • Barbara M. Ansell
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 455)


Juvenile arthritis implies an onset of disease under 16 years with arthritis persisting in one or more joints for at least six weeks, and with the active exclusion of well defined illnesses, such as systemic lupus erythematosus. Prognosis implies the ability to predict outcome. Its accuracy depends on many factors with early recognition and appropriate care being important. However, response to treatment may be variable. In general, those with involvement of a few joints do better than those with systemic disease or seropositive juvenile rheumatoid arthritis both with regard to persistence of disease activity and complications. These include not just joint deformities, but osteoporosis, amyloidosis, alterations in growth with overall failure and local anomalies, chronic iridocyclitis and psychosocial problems. More aggressive therapy was only introduced in the 1990s, so it is important that multicentre studies are properly assessed in the context of the suggested International diagnostic criteria.

One hundred years ago, George Fredric Still drew attention to the systemic form of the disease as distinct from pure polyarthritis [1], but it was only in the 1970s, as followup proceeded, that the separate identity of variants became clinically evident [2].

At the Park City meeting [3] and at the EULAR meeting in 1977 [4] when three subgroups (notably systemic, polyarthritis and pauci-articular onset) were defined, that sub-classification became regularly used. However, since there were no absolute diagnostic tests there had to be exclusions. At that time the most common medications were aspirin and corticosteroids, although a few patients received gold or penicillamine. In their large group Wallace and Levinson (1990) [5] found that at the 10 year follow-up between 31% and 55% still had active disease. Girls appeared to have a five-fold greater risk for persistent activity than boys; disease duration was probably the most important factor influencing disease activity at follow-up as suggested previously [6].

It was not until the 1990s that the more aggressive therapy in the form of methotrexate—which Giannini had shown to be effective when given in appropriate dosage

At the ILAR Meeting in 1993 an international task force was set up under the chairmanship of Dr. C. Fink [11] to develop a classification for the idiopathic arthritides in children, defining childhood as up to 16 years of age. Active exclusion of well-recognised disorders such as rheumatic fever or systemic lupus erythematosus, still had to be made. The first proposed types, which are mutually exclusive, are shown in Table 1. A more recent meeting in Durban under the chairmanship of Dr. R. Petty is yet to be published, but considerable advances have been made, particularly in the definition of subgroups.


Systemic Lupus Erythematosus Rheumatic Fever Juvenile Rheumatoid Arthritis Triamcinolone Acetonide Macrophage Activation Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    G.F. Still. On a chronic form of chronic joint disease in children. Medico-Transacter 80: 47–59 (1897).Google Scholar
  2. 2.
    B.M. Ansell, P.H.N. Wood. Progress in juvenile polyarthritis. Clin Rheum Dis 2: 297–412 (1976).Google Scholar
  3. 3.
    Proceedings of the First ARA Conference on the Rheumatic Diseases of Childhood, Park City, Utah. Arthritis Rheum, 20 (Suppl 2) (1977).Google Scholar
  4. 4.
    EULAR 1977. The Care of Rheumatic Children, (ed) E. Munthe. EULAR Publishing, Basle.Google Scholar
  5. 5.
    C.A. Wallace, J.C. Levinson. Juvenile rheumatoid arthritis: Outcome and Treatment for the 1990s. Rheum Dis Clin N Amer 17: 891–905 (1991)Google Scholar
  6. 6.
    B.M. Ansell, E.G.L. Bywaters. Progress in Still’s disease. Bull Rheum Dis 9: 189 (1959).PubMedGoogle Scholar
  7. 7.
    E.H. Giannini, E.J. Brewer, N. Kuzmina, A. Sharkon, Maximov, I.Varonstov et al. Methotrexate in resistant juvenile chronic arthritis: Results of the USA, USSR double blind placebo controlled trial. New Eng J Med 326: 1043–1049(1992)PubMedCrossRefGoogle Scholar
  8. 8.
    B.M. Ansell, M.A. Hall, J.K. Loftus, P. Woo, V. Neumann, J.A. Sills, D. Swinson, J. Insley, R. Amos, W. Dodds. A multicentre pilot study of sulphasalazine in juvenile chronic arthritis. Clin Exp Rheumatol 9: 201–203 (1991)PubMedGoogle Scholar
  9. 9.
    A. Earley, C. Cuttica, C. McCoullough, B.M. Ansell. Triamcinolone into the knee joint in juvenile chronic arthritis. Clin Exp Rheumatol 6: 153–155 (1988)PubMedGoogle Scholar
  10. 10.
    R.C. Allen, K.R. Gross, R.M. Laxer, P.N. Malesn, R.D. Beauchamp, R. Petty. Intra-articular triamcinolone hexatonide in the management of chronic arthritis in children. Arth Rheum 29: 997–1001. (1996)CrossRefGoogle Scholar
  11. 11.
    C. Fink and the Task Force. Proposal for the development of classicification criteria for Idiopathic Arthritides in Childhood. J Rheumatol 22: 1566–1569 (1995)PubMedGoogle Scholar
  12. 12.
    J.L. Stephen, J. Zeller, P. Hubert, C. Herbelin. J.M. Dayer, A.M. Prieur. Macrophage activation syndrome and rheumatic disease in childhood: a report of 4 new cases. Clin Exp Rheumatol 11: 451–456 (1993)Google Scholar
  13. 13.
    A. Ravelli, F. DeBenedetti, S. Viola, A. Martin. Macrophage activation syndrome in systemic juvenile rheumatoid arthritis successfully treated with Cyclosporin. J Pediatr 128: 275–278 (1996)PubMedCrossRefGoogle Scholar
  14. 14.
    H. Svantesson, A. Akesson, K. Eberhardt, R. Ellborgh. Prognosis in juvenile rheumatoid arthritis with systemic onset. Scand J Rheumatol 12: 139–144 (1983).PubMedCrossRefGoogle Scholar
  15. 15.
    J. David, O. Vouyiouka, B.M. Ansell, A. Hall, P. Woo. Amyloidosis in juvenile chronic arthritis: a morbidity and mortality study. Clin Exp Rheumatol 11: 85–90 (1993).PubMedGoogle Scholar
  16. 16.
    P.N. Hawkins, M.J. Myers, A.A. Eperlos, D. Caspi, M.B. Pepys. Specific localisation and imaging of amyloid deposits in vivo using 123I-labelled serum amyloid P component. J Exp Med 167: 903–913Google Scholar
  17. 17.
    P. Woo, H. Wilkes, T. Southwood, A.M Prieur, et al. Low dose methotrexate is effective in extended oligoarticular arthritis but not in systemic arthritis in children. Arthritis Rheum, Abstract 97. 1997Google Scholar
  18. 18.
    J. Loftus, R. Allen, R. Hesp, J. David, D.M. Reid, D.J. Wright, J.R. Geen. J. Reeve, B.M. Ansell, P. Woo. Randomised double-blind trial of deflazacort versus prednisone in juvenile chronic (or rheumatoid) arthritis: a relatively bone sparing effect of deflazacort. Br J Rheumatol. 32(Supp 2) 31–38 (1993).CrossRefGoogle Scholar
  19. 19.
    L. Harel, L. Wague-Weiner, A.K. Posnaski. C.H. Spencer, E. Ekoo and D.B. Magiluy. Effects of methotrexate on radiological progression in juvenile rheumatoid arthritis. Arthritis Rheum 36: 1370–1374 (1993).PubMedCrossRefGoogle Scholar
  20. 20.
    B.M. Ansell. Juvenile chronic arthritis with persistently positive tests for rheumatoid factor. Annales de Pediatrie. 30: 545–550(1983).PubMedGoogle Scholar
  21. 21.
    A.M. Rosenberg. Uveitis associated with juvenile rheumatoid arthritis. Semin Arthritis Rheum 16: 158–173 (1987).PubMedCrossRefGoogle Scholar
  22. 22.
    E. Candell Chalom, D.P. Goldsmith, M.A. Koehler, B. Bittar, C.D. Rose, B.E. Ostrov, G.F. Keenan. Prevalence and outcome of uveitis in a regional cohort of patients with juvenile rheumatoid arthritis. J. Rheumatol 24: 2035–2037(1997).Google Scholar
  23. 23.
    F. Halle, A.M. Prieur. Evaluation of methotrexate in the treatment of juvenile arthritis according to the sub type. Clin Exp Rheumatol 9: 297–302 (1991)PubMedGoogle Scholar
  24. 24.
    A.M. Rosenber, R.E. Petty. A syndrome of sero-negative enthesopathy and arthropathy in children. Arthritis and Rheum 25: 1041–1047 (1982)CrossRefGoogle Scholar
  25. 25.
    R. Brugos-Vargas, C. Pacheco-Tena, J. Vasquez-Mellado. Juvenile onset spondyloarthropathies. Rheum Dis Clin N Am 23: 569–598. (1997)CrossRefGoogle Scholar
  26. 26.
    A. Shore, B.M. Ansell. Juvenile psoriatic arthritis: an analysis of 60 patients. J. Pediatr 4: 529–535 (1982)Google Scholar
  27. 27.
    T. Southwood, R. Petty. Psoriatic arthritis in childhood. Arthritis Rheum 32: 1007–1013 (1989).PubMedCrossRefGoogle Scholar
  28. 28.
    V. Pistoia, A. Buoncompagni, R. Scribancs, L. Fasce, G. Alpigiani, G. Cordone, M. Ferrarini, C. Borrone, G. Cottafave. Cyclosporin A in the treatment of juvenile chronic arthritis and childhood polymyositis-der-matomyositis. Results of a preliminary study. Clin Exp Rheumatol 11, 203–308 (1993)Google Scholar
  29. 29.
    U.M. Davies, M. Rooney, J. Reeve, M.A. Preece, B.M. Ansell, P. Woo. Treatment of growth retardation in juvenile chronic arthritis with recombinant human growth hormone. J Rheumatol 21: 153–158 (1994).PubMedGoogle Scholar
  30. 30.
    P.H. Pepmueller, J.T. Cassidy, S.H. Alan, L.S. Hillman. Bone mineralisation and bone mineral metabolism in children with juvenile rheumatoid arthritis. Arthritis Rheum 39: 746–757 (1996).PubMedCrossRefGoogle Scholar
  31. 31.
    M.A. Muzaller, R. Schneider, R.J. Cameron, E.D. Silverman, R.M. Laxer. Accelerated nodulosis during methotrexate therapy for juvenile rheumatoid arthritis. J Pediatr 128: 698–700 (1996).CrossRefGoogle Scholar
  32. 32.
    S. Padeh, N. Sharon, G. Gchiby, G. Rechavi, J.H. Passwell. Hodgkin’s lymphoma in systemic onste juvenile rheumatoid arthritis after treatment with low-dose methrotrexate. J. Rheumatol 24: 3025–3027 (1997).Google Scholar

Copyright information

© Springer Science+Business Media New York 1999

Authors and Affiliations

  • Barbara M. Ansell
    • 1
  1. 1.Windsor BerksUK

Personalised recommendations