Clinical Case Jia

Clinical Case: Juvenile Idiopathic Arthritis
Clinical Case: Juvenile Idiopathic Arthritis (JIA)

Definition/Description
Juvenile Idiopathic Arthritis (JIA), is a chronic
inflammatory disorder that occurs before the age of 16
and can occur in all races. It may affect children at any
age, though rarely in the first six months of life. JIA causes
.persistent joint pain, swelling and stiffness
:
JIA is caused by a malfunctioning of a child’s immune system leading to inflammation of the synovial
membrane. If this inflammation persists, joint damage may occur. There are several types of arthritis all
.involving chronic joint inflammation .Inflammation is considered chronic when it has lasted at least 3 months
Pauciarticular/Oligoarthritis JIA
Polyarthritis JIA (positive RF)
Polyarthritis JIA (negative RF)
Systemic onset JIA
Psoriatic JIA
Enthesitis-related arthritis
Undifferentiated arthritis
:Typical symptoms of JIA include
Limping due to pain in lower extremities-

stiffness when awakening-
Loss of joint function (reduced range of motion/ROM)-
reluctance to use an arm of leg-
reduced activity level-
Reccurrent or persistent fever -
joint swelling-
pale or ill appearance-
difficulty with fine motor activities-
Eye irritations/inflammation (uveitis)-
Skin rash - Myalgia -
Weight loss-
. Growth problems-

Swollen right knee in JIA patient

Systemic-onset JIA is usually accompanied by an evanescent rash
(lasting a few hours), which is typically nonpruritic, macular, and salmon
colored on the trunk and extremities. Occasionally, the rash is extremely
.pruritic and resistant to antihistamine treatment
This irregularly shaped pupil is due
to synechiae between the lens and
.iris
Small joints of both h ands affected in
polyarticular JIA
Patient's may experience periods of
remission (no symptoms) and flare ups
(worsening symptoms) . Joint pain, stiffness
and swelling , More severe in the morning
. or following naps
Prevalance: - JIA occurs in children under the age of 16 and
affects more than twice in girls more than male
Girls with an oligoarticular onset outnumber boys -

.by a ratio of 3:1
.In children with uveitis, the ratio of girls to boys is 6:1-
Among children with polyarticular onset, girls to boys -
. is 2.8:1
In contrast, systemic onset occurs with equal -

-. .requency in boys and girls
The mean age of onset is 1-3 years old
:History
Arthritis
it must be present for 6 weeks before the diagnosis of (JIA)
can be made. Disease onset is either insidious or abrupt, with
morning stiffness or gelling phenomenon (ie, stiffness after long
periods of sitting or inactivity) being a frequent complaint and
.arthralgia occurring during the day
A morning limp that improves with time may be noted or after

naps .Individuals with JIA may have a history of school
absences, and their ability to participate in physical education
.classes reflects the severity of the disease or acute flares
fever-
Systemic-onset JIA is characterized by spiking

fevers, typically occurring once or twice each
day, at about the same time of day, with
temperature returning to normal or below
normal. The fever pattern is very useful
because infections, and malignancy usually
.have different pattern
- Rash
Systemic-onset JIA is usually accompanied by an
evanescent rash (lasting a few hours), which is
typically nonpruritic, macular, and salmon colored on
. the trunk and extremities
Children with psoriatic arthritis may have typical
psoriasis but dermatological manifestations may be
subtle; careful attention should be paid to looking for
nail pits. Dactylitis (swollen fingers) is characteristic
of psoriatic arthritis
back pain
Enthesitis-related arthritis frequently presents as

evening and post-exercise pain. Attention should be
given to buttock pain and back pain that improves
with activity (inflammatory back pain). These
children cannot lie in bed all morning but have to get
.up due to back pain

: Pauciarticular JIA (Oligoarthritis)
Most common type of JIA (50% of all JIA cases) -
Most common in girls younger than 8 years old- -
- . Affects less than four joints- -
Most often affects large joints (knees, elbows,-
.wrists, ankles, shoulders, hips)
. Commonly asymetrical pattern- -

Child might exhibit swollen joints and/or- -
limb/gait, most common upon waking
Leg length discrepancy common- -
Those diagnosed are 20-30% more likely to - -

. eye problems develop inflammatory
ANA (antinuclear antibodies) present - there -

is an increased risk of developing uveitis
Polyarticular JIA (PoJIA)
Makes up 30-40% of all JIA cases -
More common in girls at any age- -
Affects 5 or more joints -
Both large and small joints, but more commonly- -
[affects small joints
Commonly affects joint in a symmetrical pattern- -
PoJIA is most like adult RA - -
Two subtypes :
Rheumatoid factor positive (RF+) - patient tests ] 1
positive on at least two tests at least three months
apart . Subcutaneous nodules, cervical spine fusion,
chronic uveitis, and destructive hip disease
2]Rheumatoid factor negative (RF-) - patient tests
negative on all tests for the RF. Morning stiffness,
fatigue and possibly a low grade fever, along with
.joint involvement are typical
Systemic-onset JIA (SoJIA)
Also called Still's disease makes up 10-20% of

all JIA cases. Affects both genders equally.
Onset is most often in early childhood
.Symptoms can be present in any number of
joints and is the most severe type of JIA in that
it has many extraarticular manifestations.
Symptoms of Still's disease include
High-spiking fever - Rash on thighs and chest-
Lymphadenopathy (enlargement of lymph -

nodes) - Hepatosplenomegaly
Polyarthritis - Pericarditis (inflammation -

of the pericardium/covering of the heart)
Pleuritis (inflammation of lung -

tissues/pleura)
Peptic ulcer disease - Hepatitis-
Anemia - Anorexia - Weight loss -
Myalgia, myositis - Tenosynovitis -

Skeletal growth disturbances -
Approximately half of children with SoJIA-

almost entirely recover while one third of
children remain ill
Psoriatic JIA
Characterized by a diagnosis of arthritis and
psoriasis and at least two of the following:
inflammation and swelling of an entire finger
or toe (dactylitis), nail abnormalities, and a
.family history of psoriasis
Uveitis can be present as well -
Treated by aggressive immunosuppressives -
Enthesitis-related JIA
Characterized by inflammation of the enthesis

[(attachment of tendon, ligament, joint capsule to bone)
Enthesitis most common at Achilles tendon, spine and-

.knee
Pt diagnosed will have both arthritis and enthesitis OR-

have either arthritis or enthesitis with at least two of the
following : - Sacroiliac inflammation or pain
Spinal inflammation or pain - Positive HLA-B27 -

Positive family history of either ankylosing-
spondylitis, enthesitis- related arthritis,
inflammation of SI , inflammatory bowel
.disease, Reiter's syndrome, or acute uveitis
Acute uveitis -
Polyarticular or pauciarticular arthritis in boys -
older than 6 years
Undifferentiated arthritis
If a patient's symptoms do not fulfill the-

.criteria for any of the other six subcategories
If a patient's symptoms fulfill the criteria for-

.more than one subcategory
Complaints of joint pain may not be
predominant in the patients’ history, however;
children often stop using joints normally due
to developement of contractures of joints,
decreased wrist range, limp rather than
complain of pain. Up to a quarter of children
.with oligoarticular JIA have no pain

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Clinical Case: Juvenile Idiopathic Arthritis

Clinical Case: Juvenile Idiopathic Arthritis (JIA)

Limping due to pain in lower extremities-

Swollen right knee in JIA patient

Girls with an oligoarticular onset outnumber boys -

In contrast, systemic onset occurs with equal -

A morning limp that improves with time may be noted or after

Systemic-onset JIA is characterized by spiking

Enthesitis-related arthritis frequently presents as

.up due to back pain

Most common type of JIA (50% of all JIA cases) -

Most common in girls younger than 8 years old- -

- . Affects less than four joints- -

Most often affects large joints (knees, elbows,-

.wrists, ankles, shoulders, hips)

. Commonly asymetrical pattern- -

limb/gait, most common upon waking

Leg length discrepancy common- -

Those diagnosed are 20-30% more likely to - -

ANA (antinuclear antibodies) present - there -

Also called Still's disease makes up 10-20% of

Lymphadenopathy (enlargement of lymph -

Polyarthritis - Pericarditis (inflammation -

Pleuritis (inflammation of lung -

Myalgia, myositis - Tenosynovitis -

Approximately half of children with SoJIA-

Characterized by inflammation of the enthesis

Enthesitis most common at Achilles tendon, spine and-

Pt diagnosed will have both arthritis and enthesitis OR-

Spinal inflammation or pain - Positive HLA-B27 -

If a patient's symptoms do not fulfill the-

If a patient's symptoms fulfill the criteria for-

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