juvenile rheumatism

8/8/2019 Juvenile Rheumatism

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JUVENILE RHEUMATOID ARTHRITIS (JRA)

(JUVENILE CHRONIC ARTHRITIS-JCA)

Omondi Oyoo FACR

DIAGNOSIS AND TREATMENT


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Our task now is

not to fix theblame for the past,

but to fix the

course for the

future


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TOPICS

INTRODUCTION

EPIDEMIOLOGY

DIAGNOSIS

TREATMENT

CLINICAL OUTCOME

FUTURE

TREATMENTS


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ASSUMPTIONS

IN PREPARING TALK

AUDIENCE PREDOMINANTLY NON

RHEUMATOLOGISTS

SINCE THE TALK IS IN THE MORNING, AUDIENCE

IS PHYSICALLY STRONG AND MENTALLY ALERT

TALK WILL BE BOTH QUANTITATIVE AND

QUALITATIVE COVERING BOTH BASICS AND STRESSING KEY CONCEPTS


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JRA- Introduction

most common childhood

arthritis

common childhood

chronic illness


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JRA- Introduction

Requirements for

diagnosiscombination of data

from

history

physical examination

laboratory testing


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JRA- Introduction

Different from adult RA

inclinical course

immunogenetic

associationfunctional out come


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JRA- Introduction

5-10% JRA

rheumatoid factor

positive

poly articular

beginning in

adolescence

resembles adult

onset RA


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JRA- Epidemiology

overall 10-20 cases per 100,000

population

between 57-113/1000 childrenyounger than 16 years in the

U.S.A. (urban white)

26/100,000 urban blacks U.S.ASweden 26/100,000

Finland 18.2 cases/ 1000

population


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JRA- Epidemiology

50% of JRA have disease that

persist into adulthood

age of onset 1-3 years

girls account for majority ofpatients (twice as often as

boys)

44% concordance rate inidentical twins

4% concordance rate in

dizygotic twins


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JRA- Epidemiology

U.S.A prevalence70,000-100,000 case in a

population under 16

35,000-50,000 people over

age16 have active JRA


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JRA- Epidemiology

Comparison with other diseases

same number of children as juvenilediabetes mellitus

4 times more than sickle cell anemia

10 times more than hemophilia,

acute lymphocitic leukemia, chronic

renal failure or muscular dystrophy


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JRA- Diagnosis

AMERICAN COLLEGE OF RHEUMATOLOGY DIAGNOSTIC CRITERIA

FOR CLASSIFICATION OF JUVENILE RHEUMATOID ARTHRITIS

_____________________________________________________________

Age at onset younger than 16years

Arthritis in one or more joints defined as swelling or effusion, or the presence of

two or more of the following signs: limitation of range of motion, tenderness or pain

on motion, and increased heat

Duration of disease ofu 6 weeks

Type of onset of disease during the first 6 months classified as

-Polyarthritis- 5 joints or more

-Oligoarthrits- 4 joints or fewer-Systemic disease with arthritis and intermittent fever

Exclusion of other forms of juvenile arthritis

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JRA- Diagnosis

Four key points

arthritis

swelling effusion

limitation of motion

- tenderness

- pain on motion- joint warmth

(arthralgia is not sufficient to satisfy

this definition )


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JRA- Diagnosis

Four key points(cont)

arthritis must persist for at

least 6 weeks (ACR) (EULAR- 12weeks)

other causes of chronic arthritis

must be excluded.no specific laboratory or other

test can establish the diagnosis

of JRA


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JRA- Diagnosis

Sub division of JRA

Systemic (sJRA) 10%

Polyarticular (po JRA)

30%

Pauciarticular (pa JRA) 60%


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JRA- Diagnosis

Systemic onset JRA

(sJRA)

10% of childhood JRA

peak age 1-6 years

boys and girls equally

affected daily/twice daily

intermittent fever

characteristic JRA rash


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JRA- Diagnosis

JRA rash

pale pink

blanching characterised by small

macules on maculopapules

transient (minutes to a few

hours)

non pruritic in 95% of cases

commonly seen on the trunk


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JRA- Diagnosis

Other features of

sJRA growth delay

osteopenia

diffuse lymphadenopathy

hepatosplenomegaly pericarditis

pleuritis

anemia

leucocytosis

thrombocytosis

elevated acutephase reactants


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JRA- Diagnosis

rare features in sJRA

uveitis

positive rheumatoid

factor


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JRA- Diagnosis

complications of sJRA

pericadial tamponade

severe vasculits

prognosis determined by severity

of arthritis (may develop with feverand rash or weeks or months after

onset of fever)


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JRA- Diagnosis

Poly articular onset (po JRA)

arthritis in five or more joints

seen in 30- 40% of patients with

JRA


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JRA- Diagnosis

po JRA two distinctdiseases

RF positive

RF negative


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JRA- Diagnosis

RF positive po JRA

almost always girls later disease onset (u8 years old)

HLA DR 4 positive

symmetric small joint invovement

risk of developing nodules- erosions

- poor functional outcome

resembles adult onset RA


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JRA- Diagnosis

po JRA clinical manifestations fatigue

anorexia

growth retardation

delay in sexual maturation

osteopenia may develop at any age

girls outnumber boys 3 to 1


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JRA- Diagnosis

Pauciarticular JRA (pa JRA) arthritis in four or fever joints

two distinct clinical groups

-early onset

-late onset


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JRA- Diagnosis

Early onset pa JRA

- 1-5 years old- girls : boys 4 : 1

- ANA positive

- chronic eye inflammation (30-50% of

cases)- best overall articular outcome


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JRA- Diagnosis

Early onset pa JRA eye

involvement

- in 30 to 50% of patients- involves anteria chamber

- no or minimal symptoms in 80% of

affected children

- severe changes include- corneal clouding

- cataracts

- glaucoma

- partial or total visual loss


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JRA- Diagnosis

Late onset pa JRA

more common in boys 50% HLA-B27 positive

enthesitis/ tendinitis

arthritis large joints (shoulder hips and knees)- the spine


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JRA- Diagnosis

pa JRA

eye involvement ( rare)

- very sudden in onset

- painfull red eyes

- chronic complications less likely tooccur


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JRA- Diagnosis


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JRA- Diagnosis


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JRA- Diagnosis


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JRA- Treatment

Unique Pediatric Concerns in

treating JRA

Patients are intimate members of

family

- in house nurse, PT, OT, psychologist

- significant impact on sibs and

parental relationship- maternal depression, separation/

divorce, monetary concerns may

distract from medical care


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JRA- Treatment

Unique Pediatric Concerns in treating

JRA (cont)

Patients have a full time career- school!

Growth/nutrition must be monitored

Adolescence changes everything- denial

- sex, drugs, rock & roll

sports rule- proactive enrollment in non-contact sports

- alternative roles


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JRA- Treatment

MANAGEMENT OF JUVENILE RHEUMATIOD ARTHRITIS(cont)

Glucocorticoids

Immunosuppressive therapy

Experimentaltherapy

Orthopedic surgery

Preventive surgery

Reconstructive surgery


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JRA- Treatment

Care involves- family

- interdisciplinary health care

team


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JRA- Treatment

comprehensive care addressing- education

- peer relationship

- self esteem

- social adjustment

- family dynamics- vocational planning

- financial concerns


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JRA- Treatment

treatment

diagnosis to

satisfy- patient

- parents

- extended family

- health care team


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JRA- Treatment

theraupetic goal- relieving symptoms

- maintaining joint motion

- maintaining muscle strength

- preventing joint damage

- minimizing joint damage

- maximizing functional status- promoting positive self image

- encouraging positive/ productive

family dynamics


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JRA- Treatment

treatment programme- physical

- social

- pharmacologic

- surgical


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JRA- Treatment

physical- range of motion

exercises- splints

- joint protection

techniques


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JRA- Treatment

social

- psychosocial adjustement- school adaptation

- vocational issues


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JRA- Treatment

pharmacologic

- articular- ocular

- other manifestations


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JRA- Treatment

NSAIDS- majority respond in two weeks

- 25% do not respond until 8 to 12 weeks(average time 4 weeks)

- switch over to another NSAID if one type is

not giving good response

- Aspirin dose 75-90mg/kg/day

- Naproxen 15mg/kg/day- Ibuprofen 35mg/kg/day

- Indomethacin 2-3mg/kg/day

- Tolmetin sodium 20-30mg/kg/day


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JRA- Treatment

problems associated with

NSAIDS- anorexia

- abdominal pains

- coagulation disorders

- lever function- renal function

- cns symptoms


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JRA- Treatment

Methotrexate(MTX) and NSAIDS

- in two thirds of patients with JRA- 10mg/m2 BSA weekly

- mainly for sJRAor po JRA

- response 70-80%

non responders increase methotrexate dose to

1mg/kg/week (maximum 50mg/week)


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JRA- Treatment

problems with methotrexate- oral ulcers

- nausea

- decreased appetite

- abdominal pains

- pulmonary complications


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JRA- Treatment

oral gold 0.15mg/kg/day

d-penicillamine- 10mg/kg/day

hydroxychloroquine 5-7mg/kg/day

efficacy

similar to

placebo


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JRA- Treatment

injectible gold- 5mg test dose

- 0.75-1mg/kg weekly for 20 weeks- maintenance every 2 weeks for 3

months

- every 3 week for 3 months

- every 4 weeks- 50 60% of patients improve

- high frequency of side effects


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JRA- Treatment

sulphasalazine

- encouraging results- 40-60mg/kg/day

- A void in sJRA

intravenous gamma globulins (IVGG) promising results in po JRA


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JRA- Treatment

glucocoticoids

- used in severe and life threateningcomplications of sJRA

- used in resistant JRA

- often used in combination with other

drugs- predisone dose 0.1-1mg/kg/day


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JRA- Treatment

INDICATIONS FOR SYSTEMIC STEROIDS IN sJRA

Macrophage Activation Syndrome

CNS involvement (rare) seizures, lethargy,

meningismus

Stridor with cricoarytenoid arthritis

Interstitial pulmonary disease

Myocarditis


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JRA- Treatment

INDICATIONS FOR SYSTEMIC STEROIDS IN

sJRA(cont)

Moderate to severe pericarditis with

impairement of cardiac function

Secondary amyloidosis

Severe anemia

Failure of standard therapy to relieve

symptoms sufficiently to allow comfortable

function


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JRA- Treatment

Intraarticular steroids

in pa JRA


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JRA- Treatment

IMMUNO SUPPRESSIVE

THERAPY

chlorambucil

cyclophosphamide

azathioprime


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JRA- Treatment

IMMUNO SUPPRESSIVE THERAPY

Used in secondary amyloidosis

- life threatening illness

- un remitting progression of arthritis and

disability


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JRA- Treatment

IMMUNO SUPPRESSIVE

THERAPY

Problems

leucopenia

bone marrow supression

malignancy mutagenic effects

sterility and amenorhoea


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JRA- Treatment

eternecept

in patients refractory to or

resistant to methotrexate

dose 0.4mg/kg/dose (maximum

25mg twice weekly for threemonths)


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JRA- Treatment

Ocular

managed by experienced

ophthalmologist

- early detection

- topical corticosteroid

- dilating agents

- frequent follow up severe cases- systemic/sub-tenon steroid injection

- chlorambucil

- cyclophsophamide

- NSAIDS


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JRA- Treatment

other extra articular manifestation

- poor linear growth especially in sJRAand po JRA- growth hormone therapy

protein calorie malnutrition due to

- poor appetite

- catabolic drugs

- physical inactivity

- inflammatory medications

dietary intervention

- adequate caloric and protein intake

- nocturnal enteral nasogastric supplemental feeding


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JRA- Treatment

surgical

- synovectomy- tenosynovectomy

- re-constructive surgery


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JRA- Outcome

satisfactory for properly managed children is

approximately 85%

about 30% of JRA patients have functional

limitation after 10 or more yearsmortality 0.29 1.1 /100 patients (3 14

times greater than the standardized U.S.

children population)

ocular 85% have normal visual acuity

15% with significant visual loss

10% blinded in at least one eye.


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JRA-Future Treatments

Anti TNF -E- standard or

elevated doses

Anti IL 6Anti IL 6 + Anti TNF -E

Stem cell transplant

Pulse therapy to induceremission

- IV steroids + IV Cytoxan + MTX

Other combinations


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The very best

way to predictthe future is to

create it.


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THANK YOU

FOR YOUR

ATTENTION

juvenile rheumatism

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