Juvenile Idiopathic Arthritis and other rheumatic diseases in children

JIA and Other Rheumatic Diseases in Children Norma Liburd, RN-BC, MN Objectives Define Juvenile Idiopathic Arthritis (JIA) and discuss the diagnostic criteria Identify the subtypes of JIA and discuss characteristics of each Name at least one NSAID, one biologic and one DMARD used in the treatment of JIA A few more Objectives Discuss three school related problems students with JIA have and intervention strategies for each Identify the criteria for classification of systemic lupus erythematosus Name the most common type of juvenile localized scleroderma Discuss the criteria for diagnosis of juvenile dermatomyositis, and treatment approaches Overview of JIA New classification criteria proposed by the Pediatric Task Force of the International League of Associations for Rheumatology (ILAR) in 1997 Chronic arthritis in childhood – one of the more frequent chronic illnesses of childhood An important cause of short and long-term disability Chronic arthritis in childhood: JIA It’s not a single disease, but a group of related, genetically heterogeneous, phenotypically diverse immunoinflammatory disorders affecting joints and other structures, possibly activated by contact with an external antigen or antigens JRA - Incidence/Prevalence Published series are difficult to interpret due to classification, methodologies, heterogeneity Incidence: (per year) 1/100,000 in Japan 20/100,000 in Norway Prevalence: – – – 10 /100,000 in France 400/100,000 in Australia 113/ 100,000 Arthritis Foundation: 300,000 children in the US have chronic arthritis JRA – Classification Criteria JRA – American College of Rheumatology 1970 three types of onset: oligo (pauciarticular), polyarticular, & systemic in the first months of onset JCA Juvenile Chronic Arthritis (European League Against Rheumatism) 1977 JIA Juvenile Idiopathic Arthritis proposed by the Pediatric Task force of the International League of Associations for Rheumatology ILAR (1993) – developed to achieve homogeneity within disease and categories Sex Ratio All types of JIA: – Girls: Boys 2:1 Oligo JIA: – Girls: Boys JIA with uveitis – Girls: Boys Poly JRA: – Girls: Boys Systemic JRA: – Girls: Boys 3:1 5-6:1 3:1 approx 1:1 JIA outcomes: Mortality Disease associated death rate is < 1% in Europe < 0.3% in North America These numbers represent a Fold to 14 fold Increase in Mortality Rate Compared with General Population Causes are cardiac, infection & macrophage activation syndrome JRA outcome: functional abilities Author Year Published Followup in years (mean) Poor Function Bunim 1959 10 31% Laaksonen 1966 >16 48% Ansell 1976 >15 23% Hill 1976 (14.5) 33% Hanson 1977 5-25 (10) 28% Stoeber 1981 10-22 (15) 41% Levinson 1991 15-20 17% Zak 2000 28 11% Common NSAIDS in JIA mg/kg/day Max Naproxen 10-20 1000 Ibuprofen 30-40 2400 Indomethacin 1.5-3.0 200 Tolmetin 20-30 1800 Meloxican 0.25 15 Piroxicam 0.2-0.3 20 Celecoxib 6-12 400 Nabumetone 30 2000 (Relafen) ASA 80-100 3200 Methotrexate Standard dose: 10-15 mg/m2 or 0.3-0.6 mg/kg/week, subQ Improvement seen in 6-8 weeks, but may take up to months Labs every weeks: CBC, CMP No alcohol Used for treatment of uveitis (4-6 months to determine efficacy) Meds: Targeting inflammation Meds: Biologic Agents: Target against cytokines involved in inflammation: TNF , IL-1Ra, IL-6 Enbrel (Etanercept): approved for JRA – 0.4 mg/kg twice per week SQ injections – Improvement by third to fourth dose – Hold for suspected bacterial infection, varicella – Site reactions – Binds to TNF Biologic Agents: Remicade (Infliximab) - infusion, risk of anaphylaxis, dose may need to be increased depending on response, used in refractory uveitis as well mg/kg IV weeks 0, and (may  dose to 10 mg/kg) Improvement can be seen after first dose Labs every 4-8 weeks (CBC, CMP) Not approved for children Biologic Agents: Anakinra (Kineret) – (blocks IL-1 which stimulates synoviocytes and chondrocytes to produce small inflammatory mediators – leading to cartilage destruction and bone erosions – Used in systemic JRA (but not approved) – Daily, very painful, SQ injections, rotation of sites is important Biologics Actemra (Tocilizumab) mg/kg – ACTEMRA is indicated for the treatment of active systemic juvenile idiopathic arthritis in patients years of age and older who have responded inadequately to previously therapy with NSAIDS and steroids Given every weeks by IV, over one hour Dosing interval can be shortened to every week if condition warrants Biologics Humira (adalimumab) TNF blocker: approved for children ages to 17 Dose: 15mg (33 lbs) to