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JUVENILE RHEUMATOID ARTHRITIS

Zakiudin Munasir

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Juvenile Rheumatoid Arthritis

• Juvenile Rheumatoid Arthritis (JRA) is one of the most common rheumatic disease of childhood

• It is also one of the more frequent chronic illnesses of children and an important cause of short- and long-term disability

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JUVENILE CHRONIC (RHEUMATOID) ARTHRITIS

Definition:A chronic arthritis that persists for a minimum of six consecutive weeks in one or more joints, comencing before the age of 16 years and after active exclusion other causes. (Cassidy et al.,1989:Ansell,1990)

Systemic disease:• High remittent fever with one or more of the following-

rash, hepatomegaly, splenomegaly, generalized lymphadenopathy, serositis, usually pericarditis.

• Arthritis may be absent at the onset, but myalgia or arthralgia are usually present.

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Polyarthritic onset:• Five or more joints develop in the onset period-usually

somewhat insidiously and symmetrically• May be further divided by the presence of IgM rheumatoid

factor.Pauci-articular onset:• The commonest mode with four or fewer joints involved,

particularly knees and ankles• Two clear subgroups have emerged, notably young children

with positive antinuclear antibodies who are at risk from chronic iridocyclitis, and older boys (aged 9 upwards) who frequently carry the histocompatible leucocyte antigen (HLA) B27

• Others presenting in this way include juvenile psoriatic arthritis, the arthritis of inflammatory bowel disease and Reiter’s syndrome, while some are as yet unclassified.

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Criteria for Classification of Juvenile Rheumatoid Arthritis

1. Age at onset < 16 yr

2. Arthritis (swelling or effusion, or presence of two or more of the following signs : limitation of range of motion, tenderness or pain on motion, and increased heat) in one or more joints3. Duration of disease 6 wk or longer

4. Onset type defined by type of disease in first 6 mo:a. Polyarthritis : > 5 inflammed jointsb. Oligoarthritis (pauciarticular disease) : < 5 inflammed jointsc. Systemic-onset : arthritis with characteristic fever5. Exclusion of other forms of juvenile arthritis

Modified from Cassidy JT, Levinson JE, Bass JC, et al :A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis, 1986

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Classification:By mode of onset during the first six months (Cassidy et al.,1989:Prieur et al., 1990)

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American College of Rheumatology Guideline

“The goals of osteoarthritis (OA) management are to control pain and other symptoms, minimize disability, and educate the patient about the disease and its therapy”

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• Multidiscipline approach• Non-pharmacologic treatment• Pharmacologic treatment• Surgery

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THERAPEUTICS PRINCIPLESJuvenile Rheumatoid Arthritis

ANTIINFLAMMATORY MEDICATIONS Nonsteroidal antiinflammatory drugs COX2 inhibitors Steroids Analgesics

REHABILITATION TECHNIQUES Physical therapy Ocupational therapy Psychologic support Exercise

ADVANCED THERAPIES Immunosuppressants Biologic agents

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PHARMACOLOGIC THERAPY

1. Analgesics : acetaminophen,acetyl salicylic acid2. Nonsteroidal anti-inflammatory drugs (NSAIDs) :

The mainstay therapy of rheumatic diseases.(naproksen, ibuprofen, diklofenak)

3. Corticosteroids.4. Disease modifying antirheumatic drugs (DMARDs) :

For th/ of RA, e.g. Methotrexate, salazopyrin, hydroxychloroquine, other cytotoxics/immuno-suppressive drugs (Azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil).

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PHARMACOLOGIC THERAPY

5. Antiosteoporosis : bisphosphonate, calcitonin, 6. Biologic agents (Biologic DMARDs) :

Etanercept, infliximab etc.

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Table: Clinical Outcome-by Onset and Course SubtypesOnset Type (N) Course Subtype

(n)Profile Outcome

Polyarthritis (78)

RF seropositive (16)

FemaleOlder ageHand-wrist InvolvementErosionsNodulesUnremitting

Poor

ANA seropositive (38)

FemaleYoung age

Good

Seronegative (24) Variable Good

14ANA, anti nuclear antibodies; RF, rheumatoid factorsCassidy JT, Levinson JE, et al: A sudy of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum 29: 274-278, 1986

Table: Clinical Outcome-by Onset Type and Course Subtypes (cont.)Onset Type (N) Course Subtype (n) Profile OutcomeOligoarthritis (121)

ANA seropositive (66)

FemaleYoung ageChronic uveitis

Excellent (except eyes)

RF seropositive (8) PolyarthritisErosionsUnremitting

Poor

HLA-B27 positive (120)

MaleOlder age

Good

Seronegative (35) Variable Excellent

Systemic disease (51)

Oligoarthritis (30) Variable Good

Polyarthritis (21) Erosions poor

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Growing pains• Positive connotation : most parents accept

it as a benign condition• Negative connotation : this diagnosis has

been too frequently applied to children who actually have a serious rheumatic or malignant disease

• Most occur in preschool- to school-aged children

• The pathophysiology is unknown

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Growing Pains (Benign Nocturnal Pains of Childhood)

Age at onset 4 to 12 years

Sex ratio Probably equal, slightly more girls in some series

Symptoms Deep aching, cramping pain in thigh or calf, usually in the evening or during the night; never present in the morning; bilateral; responds to massage and analgesia

Signs Physical examination results are normal

Investigations Laboratory and radiographic studies (if done) have normal results

Modified from Cassidy JT, Petty RE, Laxer RM, Lindsley, ed. Textbook of Pediatric Rheumatology, 2005

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• Children who have unusual symptoms or abnormal findings on examination should not be diagnosed as having growing pains

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Endoscopically Detected Bleeding UlcerEndoscopically Detected Bleeding Ulcer

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