Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis Juvenile

Musculoskeletal Health in Europe Juvenile Idiopathic Arthritis

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

JIA - Incidence per 100, 000 Practitioner- or register-based study Savolainen, Finland, 2003 23 Pruunsild, Estonia, 2007 22 Berntson, Norway, Finland, Sweden, Denmark, Iceland, 2003 15 Riise, Norway, 2008 14 Hanova, Czech Republic, 2006 13 Modesto, Spain, 2010 7 Danner, France, 2006 3

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

JIA - Prevalence per Practitioner- or register-based study 100, 000 Pruunsild, Estonia, 2007 84 Hanova, Czech Republic, 2006 140 Modesto, Spain, 2010 40 Danner, France, 2006 20 Solau-Gervais, France, 2010 16

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

JIA - HLA associations JIA-category HLA-alleles conferring susceptibility Protective alleles Systemic arthritis DRB 1*04, DRB 1*11, DQA 1*05 - Oligoarthritis DRB 1*04, A 2, DRB 1*01, DRB 1*08, DRB 1*11, DRB 1*07, DRB 1*13, DPB 1*02, DQA 1*04, DQB 1*04 DQA 1*03 Seronegative Polyarthritis A 2, DRB 1*08, DQA 1*04, DPB 1*03 - Seropositive Polyarthritis DRB 1*04, DQA 1*03, DQB 1*03 DQA 1*02 Psoriatic arthritis DRB 1*01, DQA 1*0101 DRB 1*04, DQA 1*03 Enthesitis-related arthritis B 27, DRB 1*01, DQA 1*0101, DQB 1*05 -

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

JIA - Oligoarthritis Phenotype at onset: § Joints: § Function: 1 joint with arthritis 1 joint with limited range of motion CHAQ-score 0. 63 (median) § Eyes: § Lab: 4% uveitis ESR 17 mm/h, Cr. P 7 mg/l (mean) 60 -70% ANA, 0% RF

JIA - Oligoarthritis Course and outcome: After 5 years Persistent OA Extended OA 2/3 1/3 (up to 50%) 43 -84 12 -50 Functional limitations (HAQ>0), % 22 47 Frequency of erosions, % 5 33 Percentage of patients After 15 years Patients in remission, %

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 ICD 10 -Code: M 08. 3

JIA - RF negative polyarthritis Phenotype at onset: § Joints: 9 active joints and 6 with LOM § Function: CHAQ 1. 25 (median) § Eyes: § Lab: 3% uveitis ESR 22 mm/h, Cr. P 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, % Patients with functional limitations (HAQ>0), % Prevalence of erosions, % 15 -30 47 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-B 27 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 firstdegree relative

JIA - Enthesitis related arthritis Phenotype at onset: § Joints: 2 active joints and 2 with LOM § Function: CHAQ 0. 56 (median) § Eyes: § Lab: 9% uveitis ESR 10 mm/h, Cr. P 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 ICD 10 -Code: M 09. 0*, L 40. 5†

JIA - Psoriatic arthritis Phenotype at onset: § Joints: 3 active joints and 2 with LOM § Function: CHAQ 0. 56 (median) § Eyes: § Lab: 3% uveitis ESR 26 mm/h, Cr. P 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), % Patients with functional limitations (HAQ>0), % Prevalence of erosions, % 55 45 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. 2. 3. 4. Evanescent (non-fixed) erythematosus rash Generalized lymph node enlargement Hepatomegaly and/or splenomegaly Serositis

JIA - Systemic arthritis Phenotype at onset: § Joints: 2 active joints and 0 with LOM § Function: CHAQ 1. 0 (median) § Eyes: § Lab: 0% uveitis ESR 93 mm/h, Cr. P 98 mg/l (median) 15% ANA, 0% RF

JIA - Systemic arthritis Complications: Macrophage activation syndrome 1. 5 -6. 8% Growth failure Osteopenia/osteoporosis Amyloidosis 20 -41% 0 -7. 5% 1. 4 -9%

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

JIA - Systemic arthritis Course and outcome: Follow-up > 10 years Patients in remission, % Patients with functional limitations (HAQ>0), % Prevalence of erosions, % 20 -76 47 40 -75

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 ICD 10 -Code: M 08. 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, Cr. P 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) Patients (n) Biologic drugs Minden et al. Germany, 1999 JIA (215) 0% Haapasaari et al. Finland, 2000 JIA (31) 100% 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% Study Health care cost (in €) 1, 800 27, 700 (mean) (median) converted from 2000 US$ Cost components Medication: 15% Inpatient care: 55% Medication: 56% Inpatient care: n. r.

JIA - Estimated annual total cost per patient (in Euro) Functional limitation (CHAQ) moderate/severe mild none p = 0. 012 Pain intensity (NRS) 4 -10 1 -3 0 p < 0. 001 Disease activity (NRS) 4 -10 1 -3 0 p = 0. 020 0 2, 000 4, 000 6, 000 cost (€) 8, 000

JIA - mortality in children 1950 -1980 entire JIA group (90/2, 076) systemic JIA (84/611) 4% 1990 entire JIA group (33/11, 287) systemic JIA 0. 29% 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 SMR 5. 1 women (95% CI 3. 2 -7. 8) 3. 4 men (95% CI 2. 0 -5. 5)

JIA - malignancy Simard et al. , 2010, Sweden Beukelman et al. 2010, U. S. Bernatsky et al. , 2011, Canada Malignancy Standardized rate SIR n (per 100, 000 p-ys) (95% CI) JIA: 13 cases/5, 296 Controls: 30/26, 480 n. r. * 2. 3 (1. 2 -4. 4) JIA: < 11 cases/7, 321 59 2 -3 ADHS: 63/308, 454 23 Asthma: 183/23, 663 27 JIA: 1 case/1, 843 General population 4 0. 12 (0 -0. 7)

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