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Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis

Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis. Juvenile idiopathic arthritis (JIA). Definition: Juvenile idiopathic arthritis = arthritis of unknown etiology that begins before the 16th birthday and persists for at least 6 weeks.

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Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis

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  1. Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis

  2. Juvenile idiopathic arthritis (JIA) Definition: Juvenile idiopathic arthritis = arthritis of unknown etiology that begins before the 16th birthday and persists for at least 6 weeks. Other known conditions are excluded.

  3. JIA - Incidence

  4. 35% Rheumatoid factor positive polyarthritis JIA – Age at Disease Onset 30% Oligoarthritis 25% Systemic arthritis 20% Rheumatoid factor negative polyarthritis 15% Psoriatic arthritis 10% 5% Enthesitis-related arthritis 0% 0-1 2-3 4-5 6-7 8-9 10-11 12-13 14-15 Age at onset in years

  5. JIA - Prevalence

  6. Oligoarthritis JIA - Categories 52% Rheumatoid factor negative polyarthritis 16% Patients with recent onset JIA Enthesitis-related arthritis 15% Psoriatic arthritis 5% Systemic arthritis (Still´s disease) 5% Rheumatoid factor positive polyarthritis 4%

  7. JIA - HLA associations

  8. JIA - Oligoarthritis Definition: Arthritis affecting one to 4 joints during the first 6 months of disease. Two subcategories are recognized: 1. Persistent oligoarthritis: affecting not more than 4 joints throughout the disease course 2. Extended oligoarthritis: affecting a total of more than 4 joints after the first 6 months of disease

  9. Phenotype at onset: JIA - Oligoarthritis • Joints: 1 joint with arthritis 1 joint with limited range of motion • Function: CHAQ-score 0.63 (median) • Eyes: 4% uveitis • Lab: ESR 17 mm/h, CrP 7 mg/l (mean) 60-70% ANA, 0% RF

  10. JIA - Oligoarthritis Course and outcome:

  11. JIA - Rheumatoid factor negative polyarthritis Definition: Polyarthritis (Rheumatoid Factor [RF] Negative) Arthritis affecting 5 or more joints during the first 6 months of disease A test for RF is negative ICD10-Code: M08.3

  12. JIA - RF negative polyarthritis Phenotype at onset: • Joints: 9 active joints and 6 with LOM • Function: CHAQ 1.25 (median) • Eyes: 3% uveitis • Lab: ESR 22 mm/h, CrP 14 mg/l (median) 40-70% ANA, 0% RF

  13. JIA - RF negative polyarthritis

  14. JIA - RF negative polyarthritis Course and outcome:

  15. JIA - Enthesitis related arthritis Definition: Arthritis and enthesitis, or arthritis or enthesitis with at least 2 of the following: 1. The presence of or a history of sacroiliac joint tenderness and/or inflammatory lumbosacral pain 2. The presence of HLA-B27 antigen 3. Onset of arthritis in a male over 6 years of age 4. Acute (symptomatic) anterior uveitis 5. History of ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease, Reiter’s syndrome, or acute anterior uveitis in a first- degree relative

  16. Phenotype at onset: JIA - Enthesitis related arthritis • Joints: 2 active joints and 2 with LOM • Function: CHAQ 0.56 (median) • Eyes: 9% uveitis • Lab: ESR 10 mm/h, CrP 3 mg/l (median) 20% ANA, 0% RF

  17. JIA - Enthesitis related arthritis Course and outcome:

  18. JIA - Psoriatic arthritis Definition: Arthritis and psoriasis, or arthritis and at least 2 of the following: 1. Dactylitis 2. Nail pitting or onycholysis 3. Psoriasis in a first-degree relative ICD10-Code: M09.0*, L40.5†

  19. Phenotype at onset: JIA - Psoriatic arthritis • Joints: 3 active joints and 2 with LOM • Function: CHAQ 0.56 (median) • Eyes: 3% uveitis • Lab: ESR 26 mm/h, CrP 7 mg/l 45% ANA, 0% RF

  20. JIA - Psoriatic arthritis Course and outcome: 68% oligoarticular onset  65% polyarticular course

  21. JIA - Systemic arthritis Definition: Arthritis in one or more joints with or preceded by fever of at least 2 weeks’ duration that is documented to be daily (“quotidian”) for at least 3 days, and accompanied by one or more of the following: 1. Evanescent (non-fixed) erythematosus rash 2. Generalized lymph node enlargement 3. Hepatomegaly and/or splenomegaly 4. Serositis

  22. Phenotype at onset: JIA - Systemic arthritis • Joints: 2 active joints and 0 with LOM • Function: CHAQ 1.0 (median) • Eyes: 0% uveitis • Lab: ESR 93 mm/h, CrP 98 mg/l (median) 15% ANA, 0% RF

  23. JIA - Systemic arthritis Complications: Macrophage activation syndrome 1.5-6.8% Growth failure 20-41% Osteopenia/osteoporosis 0-7.5% Amyloidosis 1.4-9%

  24. Course: JIA - Systemic arthritis 42% monocyclic  remission within 2-4 years 7% relapsing  flares of systemic features and mild arthritis 51% persistent  chronic progressive polyarthritis

  25. JIA - Systemic arthritis Course and outcome:

  26. JIA - Rheumatoid factor (RF) positive polyarthritis Definition: Polyarthritis (RF positive) Arthritis affecting 5 or more joints during the first 6 months of disease 2 or more tests for RF at least 3 months apart during the first 6 months of disease are positive ICD10-Code: M08.0

  27. JIA - RF positive polyarthritis Phenotype at onset: • Joints: 9 active joints and 7 with LOM • Function: CHAQ 1.0 (median) • Eyes: 0% uveitis • Lab: ESR 15 mm/h, CrP 12 mg/l (median) 55% ANA, 100% RF

  28. JIA - RF positive polyarthritis Course and outcome:

  29. JIA - Estimated annual health care cost per patient (in Euro)

  30. JIA - Estimated annual total cost per patient (in Euro) Functional limitation (CHAQ) moderate/severe mild none p = 0.012 Pain intensity (NRS) 4-101-30 p < 0.001 Disease activity (NRS) 4-101-30 p = 0.020 0 2,000 4,000 6,000 8,000 cost (€)

  31. JIA - mortality in children 1950-1980 entire JIA group (90/2,076) 4% systemic JIA (84/611) 14% 1990 entire JIA group (33/11,287) 0.29% systemic JIA 3% 2010 entire JIA group (19/9,604) 0.2%

  32. JIA - mortality in adults  4-fold increase in mortality in JIA compared to the general population SMR5.1 women (95% CI 3.2-7.8) 3.4 men (95% CI 2.0-5.5)

  33. JIA - malignancy

  34. eumusc.net is an information and surveillance network promoting a comprehensive European strategy to optimise musculoskeletal health. It addresses the prevention and management of MSC’s which is neither equitable nor a priority within most EU member states. It is focused on raising the awareness of musculoskeletal health and harmonising the care of rheumatic and musculoskeletal conditions. It is a 3 year project that began in February 2010. It is supported by the European Community (EC Community Action in the Field of Health 2008-2013), the project is a network of institutions, researchers and individuals in 22 organisations across 17 countries, working with and through EULAR. eumusc.net: creating a web-based information resource to drive musculoskeletal health in Europe www.eumusc.net Disclaimer The Executive Agency for Health and Consumers is not responsible for any use that is made of the information contained within this publication

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