1	Modern medical treatment of JIA Rebecca ten Cate, MD PhD pediatric rheumatologist Leiden University Medical Center the Netherlands
2	format this presentation organisation of pediatric rheumatology how to deal with a child with a swollen joint introduction of a flow chart nomenclature/definitions/subsets of JIA what's new in therapy ?
3	European organisations Pediatric Rheumatology European Society www.pres.org.uk comming meeting in September in Slowakia! www.pres2004.sk Pediatric Rheumatology International Trial Organisation (trials and patient info) www.printo.it
4	Young child with a swollen knee history trauma? yes, several infection? yes, runny nose duration? ? coincidental finding physical examination hard! girl seems to have swollen knee with apperent good function consultation pediatric physiotherapist
5	non-traumatic joint complaints (less than two weeks)
6	distinghuishing steps fever CRP > 40 mg/l hips yes/no extra-articular manifestations + results of CBC/ESR and imaging
7	Retrospective analysis in 115 patients 85% directly correct diagnosis 15 % indirectly correct diagnosis in 4 children application of the flow chart would heave led to unnecessary diagnostics or therapy
8	Arthritis > 6 weeks: rheumatic, be aware of silent uveitis!
9	nomenclature Europe: juvenile chronic arthritis (JCA) USA: juvenile rheumatoid arthritis (JRA) 1997: juvenile idiopathic arthritis (JIA)
10	definition of JIA arthritis in one or more joints started before 16th birthday minimal duration of 6 weeks exclusion of known causes of arthritis!
11	Differential diagnosis infections of bone/joint reactive arthritis (rheumatic fever and PSRA) other autoimmune diseases haematological illnesses malignancies (leukaemia!) periodic fever syndromes non-rheumatic disorders pain syndromes others
12	Juvenile Idiopathic Arthritis systemic JIA (m Still) 15% polyarticular JIA 15% rheumatoid factor positive (RA) rheumatoid factor negative oligoarticular JIA eyes!! 60% persistent extended enthesitis related JIA (SPA) juvenile psoriatic arthritis (same) other arthritis
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14	Pathogenesis of JIA unknown but of autoimmune origin T-cells: HLA- association, auto-antigen unknown B-cells: auto-antibodies (ANA, RF, anti CCP) Cytokines: anti-TNFalfa
15	treatment = teamwork pediatric rheumatology physical therapy rehabilitation occupational therapy orthopaedic surgeon psychosocial workers
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18	Medical treatment NSAIDs Disease Modifying Antirheumatic Drugs hydroxychloroquine sulfasalazine Immunomodulation steroids, methotrexate anti TNF strategies ASCT
19	Glucocorticoids local eye joints oral low dose high dose pulses ………but
20	bisphosphonates
21	evidence based treatments in JIA intra-articular steroids in oligoarticular JIA sulfasalazine: good results, well tolerated but 30 % side-effects (nausea, liver) methotrexate: golden standard, especially in polyarticular subtypes etanercept
22	PRINTO-score Disease activity (visual analogue scale) ESR CHAQ pain well being number of swollen joints number of joints with limitation of movement
23	Definition of improvement 30% improvement in at least 3 of the PRINTO-criteria with no deterioration of more than 30% in one of the other criteria
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26	Anti TNF for children TNF soluble receptor (etanercept): registered for children with JIA in case of insufficient response on MTX or intolerance Lovell 2000: 75% responded without short term adverse effects better results in polyarticular JIA than in systemic JIA???
27	Autologous stem cel transplantation together with the Wilhelmina Children's Hospital in Utrecht we studied 22 children with JIA (with polyarticular course) who were therapy-resistent T-cell depleted bone marrow was reinfused after ATG, cyclophosphamide and TBI revision of the protocol after two children died of macrophage activation: less intense T-cell depletion
28	Results ASCT for therapy refractory JIA 50 % longlasting drugfree remission 30 % responding on MTX, etanercept 20 % relapse or ongoing disease activity two children died of MAS
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31	Messages a flow chart can be used in arthritis of short duration (handouts available) consult pediatric physiotherapist in longerlasting arthritis a young child with oligoarthritis should visit the ophthalmologist treatment of JIA is not essentially different from RA