RHEUMATIC

DISEASES
IN CHILDREN
Sumadiono
Department of Pediatric Fac. of Medicine Gadjah Mada University
Yogyakarta

A group of diseases in children

Rheumatic
Similar
manifestations
Diseases
Acute/chronic
inflammation
In Children

in the Musculoskeletal, Vascular, Skin

The cause is unknown
The precise diagnosis is difficult
Dianosis: by the criteria

CHILDREN with Rheumatic

Disorders

DIFFERS from ADULTS

Treatment and Outcome

Growth & Development

Emotional and Social Effects

>> 100 rheumatic disorders in

children
CLASSIFICATION

Three broad categories

Juvenile
Spondyloarthropathies

Juvenile
Rheumatoid
Arthritis

Collagen
Vascular

CLASSIFICATION

J
P
RA
o
l
yart

:
Pau
i
c
ciar ular J
ticu
RA

Syst lar JR
A
emi
c JR
A
Collagen Vascular:

Juvenile
SpondyloArthropathies:

Undifferentiated
Psoriatic Arthritis
Reactive Arthritis
Juvenile
Ankylosing
Spondylitis
Bechets
IBD

Scleroderma
Dermato myositis
SLE
Kawasaki Disease
Henoch
SchonleinPurpura
Rheumatic
Fever

CLASSIFICATION

Undifferentiated
Bechets

Dermato Scleroderma
myositis

Henoch
Juvenile
SLE
Pauciarticular Polyarticular
Schonlein
Ankylosing
JRA
Purpura
JRA
Spondylitis
Kawasaki
Psoriatic
Systemic
IBD
Disease
Arthritis
JRA
Rheumatic
Reactive
Fever
Arthritis

Disorders group with:

Chronic Synovitis

+
Extra articular manifestation
In < 16 year

JRA is the most common

class of childhood rheumatic
disease

JUVENILE RHEUMATOID
ARTHRITIS

(JRA)

JRA:
1060 Cases
/100.000
children

Female > male

Peak:
Female: 1-3 y
Male: 2-9 y
Early onset: 6
month

Limping
Stiffness of joints
on walking
Reluctance to
use a limb

ARTHRITIS:

ANKYLOSING:

SYSTEMIC MANIFESTATIONS
Hepatosplenomegali

Fever (intermitten)

Pericarditis

Pleural effusion

Spondylitis

Erytema palmaris

Rash

 Uveitis,

scleritis

rheumatoid
nodul
vasculitis

Laboratory:
Anemia
Leucocytosis, neutrophil >>.

Thrombocyte

ESR

CRP

ANA

RF

C3 & C4

IgG serum

Radiology

Early: no abnormality

Swelling soft tissue

>> joint space

Osteoporosis

New bone formation

DIAGNOSIS

JRA is defined:

Arthritis in a child </= 16 of age

In one or more joint

For six weeks or longer

Other diagnostic possibility has

been excluded

Three subtypes of JRA:

(Juvenile Idiophatic Arthritis/JIA =
JRA)
1. Pauciarticular JRA (= Oligoarticular JIA)
2. Polyarticular JRA:
Rh(+): chronic disease and Rh(-):
better
3. Systemic JRA

Extended oligoarticular JIA:

First: oligoarticular JIA (have </= 4
joints)

PAUCIARTICULAR/OLIGOARTICULAR JRA

Relative frequency
: 4045%
Extraarticular feature : Uveitis:
25%
Number of joint
: </= 4
Peak onset age (year) : 1-3 y
Female/Male ratio : 4:1
Course and prognosis:
Systemic disease absent
Major cause of morbidity is
uveitis
Prognosis excellent

POLYARTICULAR JRA

Relative frequency
45%

Number of joint
:>5
Peak onset age (year) : 1-3, >
9

: 40-

Female/Male ratio

: 5:1

Course and prognosis:

Systemic disease generally
mild
Prognosis moderately good

Extraarticular
feature:
Low grade
fever
Uveitis: 5%
Subcutaneus
nodule

SYSTEMIC JRA

Relative frequency
Number of joint
:
Peak onset age
Female/Male ratio :

: 10 %
variable
: none
1:1

Extraarticular feature
fever
Lymphadenopathy,
Hepatomegaly
Rash
Pericarditis
Course and prognosis:
Systemic & arthritis is chronic
Prognosis is variable

SPONDYLOARTHROPATHIES

Prevalence: 16.9/100,000

HISTORY:

1. Male more often

affected
2. Late onset age-ussually
> 10 years
3. Family: similar
disorder

CLINICAL SIGNS:

1. Acute iritis
2. Axial spine
involvement
3. Sacroiliits
4. Arthritis (large joint of
lower limb)

The GOALS of TREATMENT of

rheumatoid diseases in children:

Control disease activity

Maximize normal growth and development
Minimize chronic disability and deformity
Remission of the disorder

The THERAPEUTIC TEAM comprises the

following:
patient with parents / family
family practitioner / pediatrician
local pediatric hospital
pediatric rheumatologist / special hospital
orthopedist
ophthalmologist
physiotherapist
ergotherapist
social worker
Psychologist

TREATMENT of JRA

Pauciarticular JRA:
NSAID:
Aspirin: Reye Syndrome
Ibuprofen: good, no reye Syndrome
Naproxen: Standard
INTRAARTICULAR STEROID

Intra-articular Injection:
Triamcinolone Hexatomide
Lab for intra-artic fluid:
cell/WBC, culture, TB (if
needed)
Follow up: 1-2 weeks

Intra-articular Injection:

TREATMENT of JRA

Polyarticular JRA:
NSAID
Mtx orally
HydroxyChloroquine
Azathioprine

TREATMENT of JRA

Sytemic JRA:
NSAID
Steroid
Mtx
IVIG

Treatment of
Spondyloarthritis:

Steroid
Cyclosporin
Other Immunosupresive
agents

SYSTEMIC
LUPUS
ERYTHEMATOSU
S
(SLE)

Systemic Lupus Erythematosus

- a challenging disease with a wide variety
of presentations
- many systems can be affected separately

or at the same time

- occurs more commonly in girls
- considered the differential diagnosis of

many symptoms and signs

non
organ-specific
SYMPTOMS:
Fever
Fatique
Weight loss
Headache
Cognitive impairment
Psychosis, seizure
Rash
Chest pain
Shortness of breathness
Abdominal pain
Arthtralgia joint swelling

non SIGNS:
organ-specific
Alopecia, rash
Mucous membrane ulcers
Lymphadenopathy
Pleuritis, interstitiil pneum.
Pulmonary hypertention
Peri/myo/endo-carditis
Hypertension
Hepatosplenomegaly
Peripheral Vasc. Insuff.
Peripheral neuropathy
Arthritis

Malar "butterfly" rash

Mucous membane ulcers

Discoid Lupus

Vasculitis

Diagnosis: 4 + from 11 ARA

criteria

1. Eritema malar (Butterfly rash)

2. Discoid lupus
3. Photosensitivity
4. Ulseration (mucocutaneus, oral, nasal)
5. Arthritis
6. Nephritis, proteinuria>0,5 g/day
7. Ensephlophaty: convulsiion, psychosis
8. Pleuritis, pericarditis
9. Cytopenia
10. Ab. anti ds DNA +, anti Sm, LE cell
11. ANA +

TREATMENT of SLE:

Steroid
Hydroxychloroquine
Immunosupressive:
Cyclosporin
IVIG

Prognosis:
- unpredictable disease and may be fatal
- Kidney disease is one of the life
threatening
complications of SLE
- Involvement of the blood: bleeding, pallor
or
risk of infection

[HSP]
=

anaphylactoid or allergic
purpura

a small vessels vasculitis

most frequent in children between 2 and 8
years
- male:female 2:1
incidence < 15 years: 21.7/100 000
4-6 years : 70.3/100 000

Clinical manifestations:
- palpable non-thrombocytopenic purpura

- crampy abdominal pain

- and arthralgia

Clinical manifestations:
- palpable non-thrombocytopenic purpura

-crampy abdominal pain

- and arthralgia
- subcutaneous edema

Treatment
Supportive
only occasional use of corticosteroids for
the gastrointestinal complications
Complication: Renal Failure