Peadiatrics

1. Juvenile Rheumatoid
Arthritis
Presenter: Kiwano Valentino
Supervisor : Dr. Dick

2. Etiology
The chronic arthritides of childhood include several
types, the most common of which is juvenile
idiopathic arthritis (IIA). formerly called juvenile
rheumatoid arthritis (JRA). The classification of JIA
includes several other types of juvenile arthritis.
such as enthesitis-related arthritis and psoriatic
arthritis. The etiology of this autoimmune disease is
unknown. ually, the underlying bony structures.

3. The common underlying manifestation of
this group of illnesses is the presence of
chronic synovitis, or inflammation of the
joint synovium. The synovium becomes
thickened and hypervascu lar with
infiltration by lymphocytes, which also can
be found in the synovial fluid along with
inflammatory cytokines.

4. •The inflammation leads to
production and release of tissue
prote-ases and collagenases. If left
untreated, the inflammation can lead
to tissue destruction, particularly of
the articular cartilage and,eventually,
the underlying bony structure

5. EPIDEMIOLOGY
JIA is the most common chronic
rheumatologic disease of childhood, with
a prevalence of 1:1000 children. The
disease has two peaks, one at 1 to 3 years
and one at 8 to 12 years, but it can occur
at any age. Girls are affected more
commonly than boys, particularly with the
oligoarticular form of the illness.

6. CLINICAL PRESENTATION
Red eye
Limp
Arthritis
Knee pain
Extremity pain

7. • Patient with oligoarticular JIA

8. SUBTYPES
• JIA can be divided into several subtypes, depending
on the num-ber of joints involved (less than five
versus five or more), the presence of sacroiliac
involvement, and the presence of systemic
features, each with particular disease
characteristics. Although the onset of the arthritis is
slow, the actual joint swelling is often noticed
acutely by the child or parent, such as after an
accident or fall, and can be confused with trauma
(even though traumatic effusions are rare in
children).

9. • The child may develop pain and stiffness in the
joint that limit use, but rarely refuses to use the
joint at all. Morning stiffness and gelling also can
occur in the joint and, if present, can be followed
in response to therapy.
• On physical examination, signs of inflammation
are present, including joint tenderness,
erythema, and effusion. Joint range of motion
may be limited because of pain, swelling, or
contractures from lack of use. In children,
because of the

10. presence of an active growth plate, it may be
possible to find bony abnormalities of the
surrounding bone, causing bony proliferation
and localized growth disturbance. In a lower
extremity joint, a leg length discrepancy may
be appreciable if the arthritis is asymmetric.
•All children with chronic arthritis are at risk
for chronic iridocyclitis or uveitis.

11. •There is an association between
human leukocyte antigens (HLAs)
(HLA-DR5, HLA-DR6, and HLA-DR8) and
uveitis. The presence of a positive
antinuclear antibody identifies
children with arthritis who are at
higher risk for chronic uveitis.
Although all children with JIA are at

12. •increased risk, the subgroup of
children, particularly young girls,
with oligoarticular (less than five
affected joints) JIA and a positive
antinuclear antibody are at highest
risk, with an incidence of uveitis of
80%. The uveitis associated with JIA
can be asymptomatic until the point
of visual loss,

13. •making it a primary treatable cause of
blindness in children. It is crucial for
children with JIA to undergo regular
ophthalmologic screening with a slit
lamp examination to identify anterior
chamber inflammation and to initiate
prompt treatment of any active
disease.

14. Oligoarticular Juvenile Idiopathic
Arthritis
• This is defined as the presence of arthritis in
fewer than five joints within 6 months of
diagnosis. This is the most common form of JIA,
accounting for approximately 50% of cases
• Oligoarticular JIA presents in young children, with
a peak at 1 to 3 years and another peak at 8 to 12
years.

15. • The arthritis is found in medium-sized to large
joints, the knee is the most common joint involved
followed by the ankle and the wrist. It is unusual
for small joints such as the fingers or toes, to be
involved, although this may occur. Neck and hip
involvement also is uncommon.
• Children with oligoarticular JIA may be otherwise
well without any evidence of systemic inflamma
tion (fever, weight loss, or failure to thrive) or any
laboratory evidence of systemic inflammation
(elevated white blood cell count or erythrocyte
sedimentation rate). A subset of these children
later develops polyarticular disease (called
extended oligoarthritis).

16. Polyarticular Juvenile Idiopathic
Arthritis
•Polyarticular JIA describes children with
arthritis in five or more joints within the
first 6 months of diagnosis and accounts
for about 40% of cases.
•Children with polyarticular but typically
involves the small joints of the hands, feet,
ankles, JIA tend to have symmetric
arthritis, which can affect any joint wrists,
and knees The cervical spine can be
involved,

17. •Leading to fusion of the spine over
time. In contrast to oligoarticular JIA,
children with polyarticular disease can
present with evidence of systemic
inflammation, including malaise, low
grade fever, growth retardation, anemia
of chronic disease, and elevated
markers of inflammation. Polyarticular
JIA can present at any age, although
there is a peak in early childhood.

18. • There is a second peak in
adolescence, but these children differ
by the presence of a positive
rheumatoid factor (and anti-CCP
antibody) and most likely represent a
subgroup with true adult rheumatoid
arthritis; the clinical course and
prognosis are similar to the adult
entity.

19. Systemic-Onset Juvenile
Idiopathic Arthritis
• A small subgroup of patients (approximately
10%) with juvenile arthritis does not present
with onset of arthritis but rather with
preceding systemic inflammation.
• This form of JIA, thought to be an
autoinflammatory disease, manifests with a
typical recurring, spiking fever, usually once
or twice per day, which can occur for several
weeks to months.

20. •This is accompanied by a rash,
typically morbilliform and salmon-
colored. The rash may be evanescent
and occur at times of high fever only.
Rarely the rash can be urticarial in
nature. Internal organ involvement
also occurs. Serositis, such as pleuritis
and pericarditis, occurs in 50% of
children.

21. •Pericardial tamponade rarely may occur.
Hepatosplenomegaly occurs in 70% of
children. Children with systemic onset JIA
appear sick; they have significant
constitutional symptoms, including
malaise and failure to thrive.
• Laboratory findings show the
inflammation, with elevated erythrocyte
sedimentation rate, C-reactive protein,
white blood cell count, and platelet
counts and anemia.

22. •The arthritis of JIA follows the
systemic inflammation by 6 weeks
to 6 months. The arthritis is
typically polyarticular in nature and
can be extensive and resistant to
treatment, placing these children at
highest risk for long-term disability.

23. Spondyloarthropathies
•The spondyloarthropathies describe a
group of arthritides that include
inflammation of the axial skeleton and
sacroiliac joints and enthesitis, or
inflammation of tendinous insertions.
These include juvenile ankylosing
spondylitis, psoriatic arthritis, and the
arthritis of inflammatory bowel disease.

24. •This group of diseases can also present
with peripheral arthritis and can be
initially classified in other subgroups. It is
only later, when the patient develops
evidence of sacroiliac arthritis, psoriasis,
or gastrointestinal disease, that the
diagnosis becomes clear. Other important
features of this group include the
frequent presence of HLA-B27 and the
need for earlier treatment with tumor
necrosis factor (TNF) blockers.

25. LABORATORY AND IMAGING
STUDIES
• Most children with oligoarticular JIA have
no laboratory abnormalities. Children with
polyarticular and systemic-onset disease
commonly show elevated acute phase
reactants and anemia of chronic disease.
In all pediatric patients with joint or bone
pain, a complete blood count should be
performed to exclude leukemia, which
also can present with limb pain.

26. •All patients with oligoarticular JIA
should have an antinuclear
antibody test to help identify those
at higher risk for uveitis. Older
children and adolescents with
polyar-ticular disease should have a
rheumatoid factor performed to
identify children with early onset
adult rheumatoid arthritis.

27. •Diagnostic arthrocentesis may be
necessary to exclude sup-purative
arthritis in children who present with
acute onset of monarticular symptoms.
The synovial fluid white blood cell count
is typically less than 50,000 to
100,000/mm³ and should be
predominantly lymphocytes, rather
than neutrophils seen with suppurative
arthritis. Gram stain and culture should
be negative (see Chapter 118).

28. • The most common radiologic finding in the
early stages of JIA is a normal bone x-ray.
Over time, periarticular osteo-penia,
resulting from decreased mineralization, is
most commonly found. Growth centers
may be slow to develop, whereas there
may be accelerated maturation of growth
plates or evidence of bony proliferation.
Erosions of bony articular surfaces may be
a late finding. If the cervical spine is
involved, fusion of C1-4 may occur, and
atlantoaxial subluxation may be
demonstrable

29. DIFFERENTIAL DIAGNOSIS
•The diagnosis of JIA is established by
the presence of arthritis, the duration
of the disease for at least 6 weeks,
and exclusion of other possible
diagnoses. Although a presumptive
diagnosis

30. •of systemic-onset JIA can be established
for a child during the systemic phase, a
definitive diagnosis is not possible until
arthritis develops. Children must be
younger than 16 years of age at time of
onset of disease; the diagnosis of JIA
does not change when the child
becomes an adult.

31. •Because there are so many other
causes of arthritis, these disorders
need to be excluded before providing a
definitive diagnosis of JIA (Table 89-3).
The acute arthritides can affect the
same joints as JIA but have a shorter
time course.

32. Differentials
• Bacterial arthritis
• Lymphoma
• leukemia
• Sickle cell disease
• Lysosomal storage disease
• Traumatic arthritis
• Rheumatic fever
• Lyme disease

33. TREATMENT
•The treatment of JIA focuses on
suppressing inflammation, preserving
and maximizing function, preventing
deformity, and preventing blindness.
Nonsteroidal anti-inflammatory drugs
(NSAIDs) are the first choice in the
treatment of JIA. Naproxen, sulindac,
ibuprofen, indomethacin, and others
have been used successfully.

34. • Systemic corticosteroid medica-tions, such as
prednisone and prednisolone, should be avoided in
all but the most extreme circumstances, such as for
severe systemic-onset JIA with internal organ
involvement or for sig-nificant active arthritis
leading to the inability to ambulate.
• In this circumstance, the corticosteroids are used
as bridging therapy until other medications take
effect. For patients with a few isolated inflamed
joints, intra-articular corticosteroids may be
helpful.

35. •Second-line medications, such as
hydroxychloroquine and sulfasalazine, have
been used in patients whose arthritis is not
completely controlled with NSAIDs alone.
•Methotrexate, given either orally or
subcutaneously, has become the drug of
choice for polyarticular and systemic-onset
JIA, which may not respond to baseline
agents alone. Methotrexate can cause bone
marrow suppression and hepatotoxicity;
regular moni-toring can minimize these risks.

36. • Leflunomide, with a similar adverse effect profile to
methotrexate, has also been used. Bio-logic agents
that inhibit TNF-a and block the inflammatory
cascade, including etanercept, infliximab, and
adalimumab, are effective in the treatment of JIA.
• The risks of these agents are greater, however, and
include serious infection and, possi-bly, increased
risk of malignancy. Anakinra, an interleukin-1
receptor antagonist, is very beneficial in the
treatment of the systemic features of systemic-
onset JIA.

37. COMPLICATIONS
•Complications with JIA result primarily from
the loss of func-tion of an involved joint
secondary to contractures, bony fusion, or
loss of joint space. Physical and occupational
thera-pies, professionally and through home
programs, are crucial to preserve and
maximize function. More serious
complications stem from associated uveitis; if
left untreated, it can lead to serious visual
loss or blindness.

38. PROGNOSIS
•The prognosis of JIA is excellent, with an
overall 85% com-plete remission rate.
Children with oligoarticular JIA uni-
formly tend to do well, whereas children
with polyarticular disease and systemic-
onset disease constitute most children
with functional disability. Systemic-
onset disease, a positive

39. rheumatoid factor, poor response to
theratherapy and the presence of
erosions on x-ray all connote a poorer
prognosis. The importance of physical
and occupational therapy cannot be
overstated because when the disease
remits, the physical lim-itations remain
with the patient into adulthood.

40. REFERENCES
• Marlow, N. (1990). Nelson essentials of pediatrics.
Archives of Disease in Childhood, 65(10), 1186.