musculoskeletal health in europe juvenile idiopathic arthritis

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:



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


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


Phenotype at onset:



Eyes: 9% uveitis

Lab: ESR 10 mm/h, CrP 3 mg/l (median) 20% ANA, 0% RF


Course and outcome:




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†


Phenotype at onset:



Eyes: 3% uveitis

Lab: ESR 26 mm/h, CrP 7 mg/l 45% ANA, 0% RF


Course and outcome:

68% oligoarticular onset 65% polyarticular course

Outcome after > 15 years

Patients in remission off medication (> 1 year), % 55


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


Phenotype at onset:



Eyes: 0% uveitis



Complications:

Macrophage activation syndrome 1.5-6.8%Growth failure 20-41%

Osteopenia/osteoporosis 0-7.5%Amyloidosis 1.4-9%


Course:

42% monocyclic remission within 2-4 years

7% relapsing flares of systemic features and mild arthritis

51% persistent chronic progressive polyarthritis





47


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:



Eyes: 0% uveitis


JIA - RF positive polyarthritis

Course and outcome:




46


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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musculoskeletal health in europe juvenile idiopathic arthritis

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