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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
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.
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
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%
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
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
JIA - Oligoarthritis Course and outcome:
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
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
JIA - RF negative polyarthritis Course and outcome:
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
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
JIA - Enthesitis related arthritis Course and outcome:
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†
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
JIA - Psoriatic arthritis Course and outcome: 68% oligoarticular onset 65% polyarticular course
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
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
JIA - Systemic arthritis Complications: Macrophage activation syndrome 1.5-6.8% Growth failure 20-41% Osteopenia/osteoporosis 0-7.5% Amyloidosis 1.4-9%
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
JIA - Systemic arthritis Course and outcome:
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
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
JIA - RF positive polyarthritis Course and outcome:
JIA - Estimated annual health care cost per patient (in Euro)
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 (€)
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%
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)
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