Juvenile idiopathic arthritis (JIA)

JUVENILE
IDIOPATHIC ARTHRITIS
NIK NOR LIYANA BINTI NIK MD NOR SUHAIMI

INTRODUCTION
Juvenile idiopathic arthritis presents as chronic
joint swelling, pain with functional limitation
for at least 6 weeks of unknown cause that
starts before 16 year of age.
U N K N O W N
E T I O L O G Y
A T L E A S T
6 W E E K S
O N S E T
B E F O R E 1 6
Y E A R S O L D

SIGN & SYMPTOMS
A R T I C U L A R E X T R A - A R T I C U L A R
1. Joint swelling
2. Joint pain
3. Joint stiffness / gelling after
periods of inactivity
4. Joint warmth
5. Restricted joint movements
6. Limping gait
1. General
2. Growth disturbances
3. Skin
4. Others
5. Enthesitis*
*Inflammation of the entheses (the sites of insertion of tendon, ligament or joint capsule into bone)

SIGN & SYMPTOMS
E X T R A - A R T I C U L A R
General
 Fever, pallor, anorexia, loss of weight
Growth disturbances
 General : Growth failure, delayed puberty
 Local : Limb length / size decrepency, micronagthia
Skin
 Subcutaneous nodules
 Rash – systemic, psoriasis, vasculitis
Others
 Hepatomegaly, splenomegaly, lymphadenopathy
 Serositis, muscle atrophy / weakness
 Uveitis : Chronic (silent), acute in Enthesitis related arthritis
Enthesitis

CLASSIFICATION
There are 3 major types of JIA:
Some other less-common forms include:
 Enthesitis-related arthritis
 Psoriasis arthritis
 Undifferentiated arthritis
1. Oligoarticular JIA
3. Systemic JIA
2. Polyarticular JIA
RF positive
RF negative

OLIGOARTHRITIS
WHAT IS OLIGOARTHRITIS?
Oligoarticular arthritis occurs in 50% to 60% of young people who have JIA. It is
the most common type of JIA in young people. It affects four or fewer joints in
the first six months after diagnosis. There are two types of oligoarticular JIA:
1. Oligoarticular-persistent JIA
2. Oligoarticular-extended JIA.
Subtypes Age at onset Diagnostic
 Persistent
 Extended
< 6 years old  Affects ≤4 joints throughout course of disease
 Affects >4 joints after the 1st 6 months of
disease

OLIGOARTHRITIS
O L I G O A R T I C U L A R – P E R S I S T E N T A R T H R I T I S
 Mildest form of JIA.
 Affects female > male .
 It most often affects the large joints
such as the knee, ankle, wrist, and/or
elbow joints.
 It can be associated with an eye disease
called uveitis.
 It is rare to have permanent joint
damage with appropriate treatment of
this type of JIA.

OLIGOARTHRITIS
O L I G O A R T I C U L A R – E X T E N D E D A R T H R I T I S
 This type of JIA also affects four or
fewer joints in the first six months
after diagnosis. However, after six
months or more, patients with
oligoarticular-extended arthritis
develop arthritis in five or more
joints.
 Oligoarticular-extended arthritis can
affect both large and small joints.

POLYARTHRITIS
WHAT IS POLYARTHRITIS?
• The second most common type of JIA in children and teens. Affects five or
more joints within the first six months of being diagnosed with JIA.
• There are two types of polyarticular arthritis:
1. Rheumatoid factor positive polyarticular JIA, associated with the
presence of a rheumatoid factor antibody in the blood.
2. Rheumatoid factor negative polyarticular JIA, where there is no
rheumatoid factor antibody in the blood.
 RF +
 RF -
6 – 7 years
9 – 12 years
 Affects ≥5 joints in the 1st 6 months of disease
with negative RF
 Affects ≥5 joints in the 1st 6 months of disease
with ≥2 positive RF tested at least 3 months
apart

POLYARTHRITIS
P O L Y A R T I C U L A R – R F P O S I T I V E A R T H R I T I S
 Common in young people over 10 years
of age.
 Female > male
 It affects both the small joints of the
hands and feet, and large joints like the
knees, hips and ankles.
 It affects joints on both sides of the body.
 This type of JIA behaves the most like
adult rheumatoid arthritis

POLYARTHRITIS
P O L Y A R T I C U L A R – R F P O S I T I V E A R T H R I T I S
With this type of JIA, your child may also
have other symptoms:
 Rheumatoid nodules, which are hard
bumps under the skin.
 Anemia.
 Significant fatigue
 Poor appetite, with some weight loss.
 Low grade fever
 A general feeling of being unwell
These symptoms occur when the disease is
active and untreated. The symptoms will
improve with proper treatment.

POLYARTHRITIS
P O L Y A R T I C U L A R – R F N E G A T I V E A R T H R I T I S
 It can occur at any age.
 Female > male
 Usually starts in many joints at the same
time.
 Some young people only have
polyarticular JIA for a limited period of
time while others may have it for many
years.
 This type of JIA is more likely to last into
adulthood.

SYSTEMIC ARTHRITIS
WHAT IS SYSTEMIC ARTHRITIS?
Systemic arthritis is less common and affects only 10% to 15% of children and
teenagers with JIA. It is often a more severe form of JIA. Systemic means it
affects many parts of the body, rather than just the joints.
 Systemic 2 - 4 years old Affects ≥1 joints with or preceded by fever of at
least 2 weeks duration, plus ≥1 of the following:
 Transient erythematous rash
 Enlargement of lymph nodes
 Hepatomegaly
 Splenomegaly
 Pericarditis ± pleuritis ± peritonitis

SYSTEMIC ARTHRITIS
S Y S T E M I C A R T H R I T I S
 Boys = girls
 May range from mild to severe
 There is usually a spiking fever. This is a
fever that rapidly rises and falls. The
fever occurs once or twice every day.
 The JIA joint symptoms (joint pain or
swelling) begin within six months after
the fever first appears.
 Pale pink-red spots on the chest, upper
arms, thighs, and other parts of the body.
 Swollen lymph glands are common.
 Enlarged spleen and liver

SYSTEMIC ARTHRITIS
S Y S T E M I C A R T H R I T I S

ENTHESITIS ARTHRITIS
WHAT IS ENTHESITIS ARTHRITIS?
Enthesitis-related-arthritis involves inflammation in both the joints and the
entheses, which are the spots where tendons or ligaments attach to bones.
 Enthesitis
related
9 – 12 years
old
Arthritis ± enthesitis plus ≥2 of the following:
 Presence or history of sacroiliac joint
tenderness &/or inflammatory lumbosacral
pain
 Positive human leukocyte (HLA) B27 antigen
 Onset of arthritis in male >6 years old
 Acute anterior uveitis
 History of ankylosing spondylitis, enthesitis -
related arthritis, sacroilitis w/ inflammatory
bowel disease, Reiter syndrome, or family
history of acute anterior uveitis

E N T H E S I T I S A R T H R I T I S

 Male > female
 Often lasts into adulthood.
 Usually involves just a few joints in the
legs. The hips are often affected.
 Enthesitis is most common around the
knees, ankles, and bottom of the feet.
Knee, heel, and foot pain are common
with activities.
 May affect the spine and the joints
between the base of the spine and
pelvis leading to neck or back pain and
stiffness.

 Inflammation and swelling in the
tendons of the fingers and toes
making the fingers and toes look
like sausages. This is called
dactylitis.
 Inflammation of the small joints of
the feet, called tarsitis.
 Enthesitis-related arthritis may be
associated with inflammation of
the skin or bowels.

PSORIATIC ARTHRITIS
WHAT IS PSORIASIS AND PSORIATIC ARTHRITIS?
Psoriasis is a skin disease. It is a scaly red rash that can develop on the scalp,
behind the ears, on the eyelids, elbows, knees, buttocks, or in the belly button.
Some people with psoriasis may also have pits or ridges in their fingernails.
Children or teenagers with psoriasis also have arthritis. This is called psoriatic
arthritis. Sometimes the psoriasis starts before the arthritis, but sometimes the
arthritis begins before the psoriasis. A family history of psoriasis is an important
clue to the correct diagnosis.
 Psoriatic 7 – 10 years
old
Arthritis ± psoriasis plus ≥2 of the following:
 Dactylitis
 Nail pitting & oncholysis
 Family history of psoriasis

PSORIATIC ARTHRITIS
P S O R I A T I C A R T H R I T I S
 It can occur at any age.
 Male = female
 It can affect a few or many joints.
 May involve the hips or back.
 Associated with dactylitis.
 There is a moderate risk of uveitis

INVESTIGATIONS
The diagnosis is essetially clinical – labolatory investigations are only
supportive.
1. Full blood count – anaemia, leukocytosis and elevated platelets
2. ESR and peripheral blood film – markers of inflammation
3. X-ray/s of affected joint(s) - to look for malignancy
4. Antinuclear antibody – identifies risk factors for uveitis
5. Rheumatoid fever – assess prognosis in polyarthritis for early tx
6. Others
 Complement levels
 ASOT
 Ferritin
 Immunoglobulins (IgG, IgA and IgM)
 HLA B27
 Synovial fluid aspiration

TREATMENT
There is no known cure for JIA. However, there are safe and effective
medications to help control the disease. These medications help to:
1. Decrease the inflammation
2. Decrease pain and swelling
3. Make it easier for your child to stay active and exercise
4. Prevent or lessen damage to the joints.
5. Increase quality of life
NSAIDs
Corticosteroids
Corticosteroid Joint Injections
Disease Modifying Anti-Rheumatic
Drugs (MRADs)
Biologic Agents

TREATMENT
NSAIDs
Corticosteroids
Corticosteroid Joint Injections
Disease Modifying Anti-Rheumatic
Drugs (MRADs)
Biologic Agents
1. Naproxen (Naprosyn)
2. Ibuprofen (Advil)
3. Indomethacin (Indocid)
4. Diclofenac sodium (Voltaren)
1. Prednisolone
2. Methylprednisolone
1. Triamcinolone hexacetonide
2. Triamcinolone acetonide
3. Methylprednisolone
1. Methotrexate (Rheumatrex)
2. Sulfasalazine (Salazopyrin)
3. Hydroxychloroquine (Plaquenil)
4. Leflunomide (Arava)
1. Etanercept (Enbrel)
2. Infliximab (Remicade)
3. Adalimumab (Humira)
4. Rituximab (Rituxan)
5. Abatacept (Orencia)
6. Tocilizumab (Actemra)

TREATMENT
1. Physiotherapy
 Avoid prolonged immobilization
 Strengthens muscles, improves and maintain range of movement
 Improves balance and cardiovascular fitness
2. Ophthalmologist
All patients must be referred to the ophthalmologist for uveitis
screening and have regular follow-up.
3. Nutritional Therapy
 Calcium intake
 Calcium + vitamin D is advised in patients on corticosteroids
 Ensure appropriate protein and calorie intake

TREATMENT
4. Orthotics Management
 Splints and foot orthotics may be recommended
5. Thermotherapy
 Heat treatments
 Massage
 Cold treatment

REFERENCES
1. DeWitt EM, Schanberg LE, Rabiovich CE. Treatment of
Rheumatic Diseases. In: Kliegman RM, Stanton BF, St Geme
JW III, et al, eds. Nelson textbook of Pediatrics. 19th ed.
Philadelphia: Saunders, Elsevier; 2011
1. Paediatric Protocol For Malaysia Hospitals, 3rd Edition
1. https://specialty.mims.com/rheumatology
1. http://www.aboutkidshealth.ca

Juvenile idiopathic arthritis (JIA)

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