Children with Arthritis (JIA)Children with Arthritis (JIA)

Dr A Sridhar, Consultant Dr A Sridhar, Consultant Paediatrician, UHL NHS Trust, Paediatrician, UHL NHS Trust,

Leicester Leicester

Juvenile Idiopathic Arthritis (JIA)Juvenile Idiopathic Arthritis (JIA)

Most common connective tissue disease in childhoodMost common connective tissue disease in childhoodThe The DOH Musculo-skeletal frameworkDOH Musculo-skeletal framework states that states that musculo-skeletal conditions are the musculo-skeletal conditions are the biggest cause of biggest cause of disability in childrendisability in children. . In recent years, the management of children with In recent years, the management of children with Arthritis has changed significantly. Arthritis has changed significantly. The availability of the new drugs (DMARDS, Biologicals) The availability of the new drugs (DMARDS, Biologicals) has lead to a marked improvement in disease outcomeshas lead to a marked improvement in disease outcomesThe emphasis is on early diagnosis and tight disease The emphasis is on early diagnosis and tight disease controlcontrol

J.I.A.J.I.A. R.A. R.A.a disease affecting a a disease affecting a growing developing growing developing skeleton.skeleton.

a disease affecting a a disease affecting a

developed skeletondeveloped skeleton..

In children there is the capacity for regeneration, re-modelling and repairIn children there is the capacity for regeneration, re-modelling and repair

This needs to be used to a maximumThis needs to be used to a maximum

The treatment needs to be different

JIA- DefinitionJIA- Definition

Is a group of conditions Is a group of conditions

in which there is chronic arthritis in which there is chronic arthritis

lasting lasting more than 6 weeksmore than 6 weeks, ,

presenting presenting

before 16 years of agebefore 16 years of age

JIA- Criteria for DiagnosisJIA- Criteria for DiagnosisAmerican College of Rheumatology Revised American College of Rheumatology Revised

CriteriaCriteria

Age of onset < 16 yearsAge of onset < 16 years

Arthritis of one or more jointsArthritis of one or more joints

Duration of disease Duration of disease >> 6 weeks 6 weeks

Other conditions which present with Other conditions which present with arthritis in childhood must be excludedarthritis in childhood must be excluded

AetiologyAetiology

Autoimmune disease in which the cause of arthritis is Autoimmune disease in which the cause of arthritis is largely unknownlargely unknown

combination of factorscombination of factors– Environment (infection, trauma, stress)Environment (infection, trauma, stress)– Immuno-geneticImmuno-genetic

JIAJIAEpidemiologyEpidemiology

Described in all races and geographic areasDescribed in all races and geographic areasThe UK prevalence is estimated at 1:1000 under The UK prevalence is estimated at 1:1000 under 16 years with an incidence of approximately 16 years with an incidence of approximately 1:10000 1:10000 Females predominate 2:1Females predominate 2:1Leicestershire has a population of approximately Leicestershire has a population of approximately 117,600 children (Mid-2006 population 117,600 children (Mid-2006 population estimates) estimates)

JIA- JIA- Onset TypesOnset Types

Pauciarticular (Pauciarticular (<< 4 joints) 4 joints)

Polyarticular (Polyarticular (>> 5 joints) 5 joints)

Systemic (arthritis with fever and rash)Systemic (arthritis with fever and rash)

ILAR Proposed Classification CriteriaILAR Proposed Classification Criteria

Juvenile Idiopathic Arthritis (JIA)Juvenile Idiopathic Arthritis (JIA)SystemicSystemicPolyarticular: RF+ and RF-Polyarticular: RF+ and RF-Oligoarticular: persistent and extendedOligoarticular: persistent and extendedPsoriatic arthritisPsoriatic arthritisEnthesitis-related arthritisEnthesitis-related arthritis

Presenting features of Arthritis Presenting features of Arthritis

Joint PainJoint Pain

Joint StiffnessJoint Stiffness

Joint swellingJoint swelling

LimpLimp

Restriction of movementRestriction of movement

Eye symptoms Eye symptoms

Systemic symptoms Systemic symptoms

Common Differential DiagnosisCommon Differential Diagnosis

Irritable hip- Transient SynovitisIrritable hip- Transient Synovitis

Septic Arthritis, OsteomyelitisSeptic Arthritis, Osteomyelitis

Infection – Viral, Bacterial, Lyme diseaseInfection – Viral, Bacterial, Lyme disease

Malignancy – leukaemia, NeuroblastomaMalignancy – leukaemia, Neuroblastoma

Perthe’s Disease Perthe’s Disease

Slipped Capital Femoral Epiphysis (SCFE)Slipped Capital Femoral Epiphysis (SCFE)

Generalised Joint and Bone painsGeneralised Joint and Bone pains

Non-specific but clinically well, No joint Non-specific but clinically well, No joint SwellingSwelling

Hyper-mobility of Joints- either Syndromic Hyper-mobility of Joints- either Syndromic or Benign Hypermobilty syndromesor Benign Hypermobilty syndromes

Vitamin D deficiencyVitamin D deficiency

JIAJIAManagementManagement

No cure but treatable No cure but treatable

Remissions and Relapses Remissions and Relapses

Involves multidisciplinary team (MDT) Involves multidisciplinary team (MDT) approachapproach

Relieve pain, reduce inflammation, Relieve pain, reduce inflammation, preserve joint function, maintain normal preserve joint function, maintain normal growth and developmentgrowth and development

Screen for Uveitis Screen for Uveitis

Medical ManagementMedical Management

NSAIDS – Ibuprofen, Naproxen, Piroxicam NSAIDS – Ibuprofen, Naproxen, Piroxicam DMARDS (Disease modifying anti rheumatic drugs)DMARDS (Disease modifying anti rheumatic drugs)

- under Rheumatologist’s supervision- under Rheumatologist’s supervision

- Methotrexate: Orally or subcutaneously: Weekly- Methotrexate: Orally or subcutaneously: Weekly

Joint steroid injectionsJoint steroid injectionsCorticosteroids : Oral or IV Methyprednisolone Corticosteroids : Oral or IV Methyprednisolone Anti- TNF agents Anti- TNF agents

– – block the immune protein TNF : Infliximab, Etarncept, block the immune protein TNF : Infliximab, Etarncept, Adalimumab Adalimumab

Physiotherapist & Occupational Physiotherapist & Occupational TherapistsTherapists

Develop exercise programsDevelop exercise programs

Strengthen muscles & keep joints flexibleStrengthen muscles & keep joints flexible

Encourage normal limb developmentEncourage normal limb development

Maintain function and prevent deformitiesMaintain function and prevent deformities

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JIAJIAPrognosisPrognosis

Chronic disease which is treatable but cannot be Chronic disease which is treatable but cannot be curedcured

Characterized by remissions and Relapses Characterized by remissions and Relapses

Overall the prognosis is much better in JIA Overall the prognosis is much better in JIA compared to RAcompared to RA

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISPoor Prognostic SignsPoor Prognostic Signs

PauciarticularPauciarticular– Long duration of active diseaseLong duration of active disease– Conversion to polyarticular disease Conversion to polyarticular disease – Chronic uveitisChronic uveitis

PolyarticularPolyarticular– Long duration of active diseaseLong duration of active disease– Articular erosionsArticular erosions– RF positivityRF positivity

SystemicSystemic– Conversion to polyarticular disease Conversion to polyarticular disease

Paediatric Rheumatology Service Paediatric Rheumatology Service Aims of the Service Aims of the Service

To provide a high quality care for children To provide a high quality care for children and young people with Rheumatological and young people with Rheumatological problems locally problems locally

To provide or enable them to receive the To provide or enable them to receive the care at home care at home

Paediatric Rheumatology Service Paediatric Rheumatology Service

Running the service:15 yrs Running the service:15 yrs

All children < 16 yrs ageAll children < 16 yrs age

Children between 16-18 yrs( full time Children between 16-18 yrs( full time education) or with other chronic medical education) or with other chronic medical conditions conditions

Significant number of children with JIA on Significant number of children with JIA on Methotrexate therapy Methotrexate therapy

Clinical team Clinical team

Consultant Paediatricians with specialist Consultant Paediatricians with specialist interest in Paediatric Rheumatology interest in Paediatric Rheumatology

Adult Rheumatologist Adult Rheumatologist

Specialist RegistrarSpecialist Registrar

Clinical Nurse Specialist (CNS)Clinical Nurse Specialist (CNS)

Conditions seen Conditions seen

JIA and other Inflammatory Arthropathies JIA and other Inflammatory Arthropathies

Non-inflammatory Musculo-skeletal pain Non-inflammatory Musculo-skeletal pain syndromessyndromes

Childhood Vasculitis Childhood Vasculitis

Hyper-mobility syndromes Hyper-mobility syndromes

OPD clinicsOPD clinics

OPD Clinics; New and follow up OPD Clinics; New and follow up

Consultant/Registrar ledConsultant/Registrar led

Weekly clinics Weekly clinics

Rapid access clinics Rapid access clinics

Annual ReviewsAnnual Reviews

Children’s Day Care Unit Children’s Day Care Unit

Mon-Fri, 0800- 1800 hrs Mon-Fri, 0800- 1800 hrs Methotrexate info, counselling , injections, Methotrexate info, counselling , injections, training to parents training to parents Bloods sampling Bloods sampling Joint steroid injections Joint steroid injections Specialist Nurse support- Close liaison with Specialist Nurse support- Close liaison with Diana team in the community Diana team in the community Methotrexate MDT meetings: Methotrexate MDT meetings:

( 2 meetings/month): Specialist nurse, ( 2 meetings/month): Specialist nurse, Pharmacist and Consultant Pharmacist and Consultant

Inpatient ServicesInpatient Services

Dedicated support by on-call teamDedicated support by on-call team

Open access to Children’s assessment Open access to Children’s assessment unit- 24x 7unit- 24x 7

Multi-disciplinary team: Physiotherapy, Multi-disciplinary team: Physiotherapy, OT, other speciality input OT, other speciality input

Support ServicesSupport Services

Paediatric Physiotherapist Paediatric Physiotherapist

Occupational Therapist (O.T)Occupational Therapist (O.T)

Podiatrist/OrthotistPodiatrist/Orthotist

Paediatric PharmacistPaediatric Pharmacist

Paediatric Dietician Paediatric Dietician

Play therapist Play therapist

Dedicated secretarial support Dedicated secretarial support

Specialist ServicesSpecialist Services

Paediatric Ophthalmologist Paediatric Ophthalmologist

Paediatric Dermatologist Paediatric Dermatologist

Paediatric Orthopaedic SurgeonPaediatric Orthopaedic Surgeon

Paediatric Radiologist: X-rays, U/S, MRI Paediatric Radiologist: X-rays, U/S, MRI ScansScans

Musculo-Skeletal Radiologist; Monthly Musculo-Skeletal Radiologist; Monthly meetingsmeetings

Transition Transition

Transitional Services Transitional Services