is an umbrella term referring to a group of disorders characterized by chronic arthritis. JIA is the most common chronic rheumatic illness in children and is a significant cause of short-and long-term disability. It is a clinical diagnosis made in a child less than16 years of age with arthritis (defined as swelling or limitation of motion of the joint accompanied by heat, pain, or tenderness) for at least 6 weeks’ duration with other identifiable causes of arthritis excluded.

1. rawand
2019

2. Juvenile idiopathic arthritis (JIA)
is an umbrella term referring to a group of disorders
characterized by chronic arthritis. JIA is the most
common chronic rheumatic illness in children and is
a significant cause of short-and long-term disability.
It is a clinical diagnosis made in a child less than16
years of age with arthritis (defined as swelling or
limitation of motion of the joint accompanied by heat,
pain, or tenderness) for at least 6 weeks’ duration
with other identifiable causes of arthritis excluded.

4.  There are significant differences in the disease
manifestations in children compared with adults,
with some types occurring exclusively in children.
 JIA affects at least 1 in 1000 children.
 Juvenile idiopathic arthritis affects a much smaller
portion of the US population than adult-onset RA.
 JIA is relatively common, affecting approximately the
same number of children as juvenile diabetes, at least
four times as many children as sickle cell anemia or
cystic fibrosis, and at least 10 times as many as
hemophilia, acute lymphocytic leukemia, chronic
renal failure, or muscular dystrophy.

5.  JRA Juvenile rheumatoid arthritis :
( American College of Rheumatology, 1977)
 JCA Juvenile Chronic Arthritis :
(European League Against Rheumatism,
1978)
 JIA Juvenile Idiopathic Arthritis :
(The International League of Associations for
Rheumatology ILAR ,1997)

6.  There is evidence of immuno-dysregulation in JIA.
Complement activation and consumption promote
inflammation, and increasing serum levels of
circulating immune complexes are found with
active disease.
 Anti-nuclear antibodies (ANA) are found in
approximately 40% of patient’s with JIA , especially
in young girls with pauciarticular disease.
Approximately5% to10% of patients with JIA are RF
positive.
 The T-lymphocyte–mediated immune response is
involved in chronic inflammation, and T cells are
the predominant mononuclear cells in synovial
fluid.

7.  Patients with JIA have elevated serum levels of
interleukin (IL)-1, -2, -6,and IL-2 receptor (R) and
elevated synovial fluid levels of IL-1b, IL-6, andIL-
2R, suggesting a Th1 profile. Elevated serum levels
of IL-6, IL2R,and soluble tumor necrosis factor
(TNF) receptor correlate with inflammatory
parameters, such as C-reactive protein, in JIA
patients with active disease. Se-rum levels of IL-6
are increased in SOJIA and rise before each fever
spike, correlating with active disease and elevation
of acute-phase reactants.

8. SIGN & SYMPTOMS
A R T I C U L A R
Joint swelling
Joint pain
Joint stiffness / gelling after
periods of inactivity
Joint warmth
Restricted joint movements
Limping gait

9. 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

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

11. Classification of JIA

12. Classification of JIA

14. Oligoarticular JIA
 Arthritis affecting 1-4 joints during the first 6 months
of disease. Oligoarticular arthritis counts for 30% to
60% of all JIA. It is the most common type of JIA.
 Oligoarticular JIA usually manifests as an asymmetric
arthritis affecting one or two large joints, especially of
the lower extremities, with the knee the most
commonly affected, followed by the ankle, wrist, and
digits. Involvement of the hip and back, especially in
young children, is so unusual that extensive evaluation
is warranted to rule out other conditions such as
infection or malignancy.
 Significant constitutional and systemic symptoms are
unusual in oligoarticular JIA and, if present, should
raise concern regarding the accuracy of the initial
diagnosis.

15. Oligoarticular JIA
Among children with oligoarticular JIA, a positive ANA in
low to moderate titers (1 : 40 to 1 : 320) is seen in 70% to
80% of children with persistent oligoarticular JIA and
80% to 95% with extended oligoarticular JIA. The rate of
ANA positivity is even higher in girls with early onset
disease. The typical child with oligoarticular JIA will have
normal white blood counts, normal or mild-moderately
elevated acute phase reactants, and, in some cases, mild
anemia.
Two subcategories are recognized:
1. Persistent oligoarticular JIA: affecting ≤4 joints
throughout the disease course.
2. Extended oligoarticular JIA : affecting a total of >4
joints after the first 6 months of disease.

16. 1. Persistent oligoarticular JIA:
 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.

17. 2. Extended oligoarticular JIA :
 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.

19. Oligoarticular JIA
Exclusions
a) Psoriasis or a history of psoriasis in the patient or
a first-degree relative
b) Arthritis in a human leukocyte antigen (HLA)-
B27+ male beginning after the sixth birthday
c) Ankylosing spondylitis, ERA, sacroiliitis with
inflammatory bowel disease, Reiter’s syndrome, or
acute anterior uveitis, or a history of one of these
disorders in a first-degree relative.
d) The presence of IgM RF on at least 2 occasions, at
least 3 months apart
e) The presence of systemic JIA in the patient

20. RF− polyarticular JIA
 Arthritis affecting ≥5 joints during the first 6months of
disease, and Test for RF is negative.
 6 – 7 years.
 It can occur at any age.
 Female > male
 Usually starts in many joints at the same time.
 Some time 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.

21. RF− polyarticular JIA
Exclusions :
a) Psoriasis or a history of psoriasis in the patient or
a first-degree relative.
b) Arthritis in a human leukocyte antigen (HLA)-
B27+ male beginning after the sixth birthday.
c) Ankylosing spondylitis, ERA, sacroiliitis with
inflammatory bowel disease, Reiter’s syndrome,or
acute anterior uveitis, or a history of one of these
disorders in a first-degree relative.
d) The presence of IgM RF on at least 2 occasions, at
least 3 months apart.
e) The presence of systemic JIA in the patient.

22. RF+ polyarticular JIA
 Arthritis affecting ≥5 joints during the first 6
months of disease, and ≥2 positive RF tests
(as routinely defined in an accredited
laboratory), at least 3 months apart during
the first 6 months of disease.
 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.

23. RF+ polyarticular
 Exclusions :
a) Psoriasis or a history of psoriasis in the patient or a
first-degree relative.
b) Arthritis in a human leukocyte antigen (HLA)-B27+
male beginning after the sixth birthday.
c) Ankylosing spondylitis, ERA, sacroiliitis with
inflammatory bowel disease, Reiter’s syndrome,or
acute anterior uveitis, or a history of one of these
disorders in a first-degree relative.
d) The presence of systemic JIA in the patient.

24. Psoriatic arthritis
1) Arthritis and psoriasis, or
2) Arthritis and at least 2 of the following:
 Dactylitis.
 Nail pitting (minimum of 2 pits on ≥1 nails at any
time) or onycholysis.
 Psoriasis in a first-degree relative.
 7 – 10 years old
 Sometimes the psoriasis starts before the arthritis, but
sometimes the arthritis begins before the psoriasis.

25. Psoriatic arthritis
 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

26. Exclusions
a) Arthritis in a human leukocyte antigen (HLA)-
B27+ male beginning after the sixth birthday
b) Ankylosing spondylitis, ERA, sacroiliitis with
inflammatory bowel disease, Reiter’s
syndrome, or acute anterior uveitis, or a
history of one of these disorders in a first-
degree relative.
c) The presence of IgM RF on at least 2 occasions,
at least 3 months apart
d) The presence of systemic JIA in the patient

27. Enthesitis-related arthritis
1) Arthritis and enthesitis, or
2) Arthritis or enthesitis, with at least 2 of the following:
a) The presence of or a history of sacroiliac joint
tenderness and/or inflammatory lumbosacral pain
b) The presence of HLA-B27.
c) Onset of arthritis in a male >6 years of age.
d) Acute (symptomatic) anterior uveitis.
e) History of ankylosing spondylitis, ERA, sacroiliitis
with inflammatory bowel disease, Reiter’s
syndrome, or acute anterior uveitis in a first-
degree relative.

28. Enthesitis-related arthritis
 9 – 12 years old
 Enthesitis-related-arthritis involves inflammation in
both the joints and the entheses, which are the
spots where tendons or ligaments attach to bones.
 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.

30. Enthesitis-related arthritis
 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.

31. Enthesitis-related arthritis
Exclusions
a) Psoriasis or a history of psoriasis in the patient or a
first-degree relative
b) The presence of IgM RF on at least 2 occasions, at
least 3 months apart
c) The presence of systemic JIA in the patient

32. Systemic JIA
 Arthritis in ≥1 joints with, or preceded by,
fever of at least 2 weeks’ duration that is
documented to be daily and quotidian
(fever that rises to ≥39° C once a day and
returns to ≤37° C between fever peaks) for
at least 3 days, and accompanied by ≥1 of
the following:
a. Evanescent (non-fixed) erythematous rash.
b. Generalized lymph node enlargement.
c. Hepatomegaly and/or splenomegaly.
d. Serositis.

33. Systemic JIA
 2 - 4 years old
 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.
 Boys = girls
 May range from mild to severe
 here 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

34. Systemic JIA

35. Systemic JIA

36. Systemic JIA

37. Systemic JIA
Exclusions :
a) Psoriasis or a history of psoriasis in the patient or a
first-degree relative.
b) Arthritis in a human leukocyte antigen (HLA)-B27+
male beginning after the sixth birthday.
c) Ankylosing spondylitis, ERA, sacroiliitis with
inflammatory bowel disease, Reiter’s syndrome,or
acute anterior uveitis, or a history of one of these
disorders in a first-degree relative.
d) The presence of IgM RF on at least 2 occasions, at least
3 months apart.

38. Undifferentiated arthritis
Arthritis that fulfills criteria in no category
or in ≥2 of the other categories.

39. 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

40. ACR and Arthritis Foundation Release New
Treatment Guidelines for Juvenile Arthritis.(2019)
A number of treatments are available, including
nonsteroidal anti-inflammatory drugs (NSAIDs), systemic
and intraarticular glucocorticoids, and non-biologic and
biologic disease-modifying anti-rheumatic drugs
(DMARDs).
Prompt initiation of appropriate therapy is of critical
importance in preventing permanent damage and
improving outcomes. While earlier diagnosis and
expanded treatment options have improved disease
control, For JIA polyarthritis, favors initial therapy with
DMARDs over NSAIDs, given as a single therapy.
NSAIDs or intraarticular glucocorticoids are conditionally
recommended as an adjunct (add-on) treatment, based
on the very low quality of evidence supporting its use
along with patient and caregiver preferences, and
concerns regarding adverse side effects.

41.  Starting treatment with a combination of a biologic
therapies, such as etanercept (sold as Enbrel),
adalimumab (Humira), golimumab (Simponi),
abatacept (Orencia), or tocilizumab (Actemra), and
a DMARD also is conditionally recommended over
the use of biologic agents alone.
 There is strong recommendation against adding
low-term, low-dose glucocorticoids, irrespective of
risk factors or disease activity. This decision was
supported by the known adverse effects of this
regimen in children, particularly growth
suppression, weight gain, loss of bone mass, and
cataracts (clouding of the eye’s lens).

42.  For young patients at risk for functional
limitations, it is recommended to get
physical therapy and/or occupational
therapy. This recommendation is conditional
considering the low quality of evidence
supporting a benefit for such interventions.
 It also support relatively tight disease
control, with inactive disease as the goal.
While it is anticipated that these
recommendations will lead to improved
outcomes for children with JIA and these
phenotypes.

43.  The second guideline released important
recommendations for the management of JIA-
associated uveitis. Chronic inflammation of the eye,
medically identified as chronic anterior uveitis
(CAU), affects about 10-20% of JIA children, and
acute anterior uveitis (AAU) typically occurs in
children with spondyloarthritis — meaning those
with enthesitis or psoriatic arthritis.
 For this group of patients there is strongly
recommended a more frequent ophthalmologic
monitoring, particularly for those with controlled
uveitis. This is specifically important to be done
within one month after each change of topical
(applied directly) glucocorticoids, within two months
for those who are tapering or discontinuing systemic
therapy (non-biologic DMARDs and biologic
therapies), and at least every three months for
patients on stable therapy.

44.  For children and adolescents with severe, active CAU and
sight-threatening complications, there is a conditional
recommendation to immediately start methotrexate (a
DMARD agent) plus one of the available anti-TNF biologics
— infliximab (sold as Remicade, Flixabi, among others) or
adalimumab —rather than methotrexate alone. This
recommendation was conditional given the lack of direct
evidence from clinical studies, the risk of permanent vision
loss, and anticipated differences in patient values and
preferences.
 In addition, the guidelines strongly recommend educating
JIA patients with spondyloarthritis regarding the warning
signs of AAU so that it is possible to reduce delays in
treatment, duration of symptoms, or complications of iritis
(inflammation of the colored structure of the eye).
 Prevention of sight-threatening complications from uveitis is
most important. It is crucial that children with JIA undergo
scheduled ophthalmology screening to detect uveitis early
since children are usually asymptomatic.

45. Treatment
 NSAIDs
 Naproxen (Naprosyn)
 Ibuprofen (Advil)
 Indomethacin (Indocid)
 Diclofenac sodium (Voltaren)
 Corticosteroids
 Prednisolone
 Methylprednisolone
 Corticosteroid Joint
Injections
 Triamcinolone hexacetonide
 Triamcinolone acetonide
 Methylprednisolone
 Disease Modifying Anti-
Rheumatic
 Methotrexate (Rheumatrex)
 Sulfasalazine (Salazopyrin)
 Hydroxychloroquine
(Plaquenil)
 Leflunomide (Arava)
 Biologic Agents
 Etanercept (Enbrel)
 Infliximab (Remicade)
 Adalimumab (Humira)
 Rituximab (Rituxan)
 Abatacept (Orencia)
 Tocilizumab (Actemra

50. 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

51. Thank you