musculoskeletal health in europe juvenile idiopathic arthritis
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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. - PowerPoint PPT PresentationTRANSCRIPT
Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis
Juvenile idiopathic arthritis
(JIA)
Definition:
Juvenile idiopathic arthritis = arthritisof unknown etiology
that begins before the 16th birthdayand persists for at least 6 weeks.
Other known conditions are excluded.
JIA - Incidence
JIA – Age at Disease Onset
0-1 2-3 4-5 6-7 8-9 10-11 12-13 14-15
0%
5%
10%
15%
20%
25%
30%
35%
Systemic arthritis
Psoriatic arthritis
Enthesitis-related arthritis
Rheumatoid factor negative polyarthritis
Age at onset in years
Oligoarthritis
Rheumatoid factor positive polyarthritis
JIA - Prevalence
JIA - Categories
Oligoarthritis
Rheumatoid factor negative polyarthritis
Enthesitis-related arthritis
Psoriatic arthritis
Systemic arthritis (Still´s disease)
Rheumatoid factor positive polyarthritis
52%
16%
15%
5%
5%
4%
Patients with recent onset
JIA
Patients with recent onset
JIA
JIA - HLA associations
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
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
Phenotype at onset:
JIA - Oligoarthritis
JIA - Oligoarthritis
Course and outcome:
After 5 years Persistent OA Extended OA
Percentage of patients 2/3 1/3 (up to 50%)
After 15 years
Patients in remission, % 43-84 12-50
Functional limitations (HAQ>0), % 22 47
Frequency of erosions, % 5 33
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
JIA - RF negative polyarthritis
Course and outcome:
Follow-up > 10 years
Patients in remission, % 15-30
Patients with functional limitations (HAQ>0), %
47
Prevalence of erosions, %40-75
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 antigen3. Onset of arthritis in a male over 6 years of age4. Acute (symptomatic) anterior uveitis5. History of ankylosing spondylitis, enthesitis related
arthritis, sacroiliitis with inflammatory bowel disease, Reiter’s syndrome, or acute anterior uveitis in a first- degree relative
JIA - Enthesitis related arthritis
Phenotype at onset:
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:
Follow-up > 10 years
Patients in remission, % 17-44
Patients with functional limitations (HAQ>0), %
47
Prevalence of sacroiliits, %35
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†
JIA - Psoriatic arthritis
Phenotype at onset:
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
Outcome after > 15 years
Patients in remission off medication (> 1 year), % 55
Patients with functional limitations (HAQ>0), %
45
Prevalence of erosions, %23
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 rash2. Generalized lymph node enlargement3. Hepatomegaly and/or splenomegaly4. Serositis
JIA - Systemic arthritis
Phenotype at onset:
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%
JIA - Systemic arthritis
Course:
42% monocyclic remission within 2-4 years
7% relapsing flares of systemic features and mild arthritis
51% persistent chronic progressive polyarthritis
JIA - Systemic arthritis
Follow-up > 10 years
Patients in remission, % 20-76
Patients with functional limitations (HAQ>0), %
47
Prevalence of erosions, %40-75
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:
Follow-up > 10 years
Patients in remission, % 0-46
Patients with functional limitations (HAQ>0), %
46
Prevalence of erosions, %39-77
JIA - Estimated annual health care cost per patient (in Euro)
StudyPatients
(n)Biologic drugs
Health care cost (in €) Cost components
Minden et al.Germany, 1999
JIA(215)
0% 1,800 (mean)Medication: 15%Inpatient care: 55%
Haapasaari et al. Finland, 2000
JIA (31)
100%27,700 (median)
converted from 2000 US$
Medication: 56%Inpatient care: n.r.
Minden et al,Germany, 2003
JIA (369)
6% 4,200 (mean)Medication: 47%Inpatient care: 39%
Thornton et al.U.K., 2005
JIA(297)
n.r. 2,400 (mean)Medication: 11%Inpatient care: 0%
JIA - Estimated annual total cost per patient (in Euro)
0 2,000 4,000 6,000 8,000
cost (€)
Functional limitation (CHAQ)Functional limitation (CHAQ)
Disease activity (NRS)Disease activity (NRS)
Pain intensity (NRS)Pain intensity (NRS)
moderate/severe mild
none
4-101-3
0
4-101-3
0
p = 0.012p = 0.012
p < 0.001 p < 0.001
p = 0.020 p = 0.020
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%
2010entire JIA group (19/9,604) 0.2%
JIA - mortality
SMR 5.1 women (95% CI 3.2-7.8) 3.4 men (95% CI 2.0-5.5)
4-fold increase in mortality in JIA compared to the general population
in adults
JIA - malignancy
Malignancy n
Standardized rate (per 100,000 p-ys)
SIR(95% CI)
Simard et al., 2010, Sweden
JIA: 13 cases/5,296Controls: 30/26,480
n.r.*n.r.*
2.3(1.2-4.4)
Beukelman et al. 2010, U.S.
JIA: < 11 cases/7,321 59 2-3
ADHS: 63/308,454 23
Asthma: 183/23,663 27
Bernatsky et al., 2011, Canada
JIA: 1 case/1,843General population
4 0.12(0-0.7)
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