Overview of
Juvenile Idiopathic
Arthritis
Pediatric Rheumatology
Red Team Resident
Teaching Series
Juvenile Idiopathic Arthritis
• One of the more common chronic
childhood diseases
• almost as common as childhood-onset diabetes
mellitus
• at least 4 times more common than sickle cell disease
or cystic fibrosis
• at least 10 times more common than leukemia,
hemophilia, chronic renal failure, or muscular
dystrophy
Differential Diagnosis for JIA
• Acute (within 72 hours)
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Infection: Septic arthritis, Rheumatic fever
Malignancy: Leukemia, Neuroblastoma
Toxic Synovitis
Blood disorders: Hemophilia, Sickle Crisis (dactylitis)
Trauma
• Chronic (more than 4 weeks)
• Infection: TB, Lyme disease, Parvovirus, Rubella
• Pigmented villonodular synovitis (PVNS)
• Other rheumatic diseases (SLE, Sarcoid, vasculitis)
CRITERIA FOR THE DIAGNOSIS OF
JUVENILE IDIOPATHIC ARTHRITIS
• Age of onset < 16 years
• Arthritis (swelling or effusion, limitation of motion,
tenderness or pain on motion, and/or increased
heat) in one or more joints
• Duration of disease 6 weeks or longer
• Onset type defined by type of disease in the first 6
months:
Polyarticular: 5 or more inflamed joints
Oligoarticular: < 5 inflamed joints
Systemic arthritis: Characteristic fever
• Exclusion of other forms of arthritis
Juvenile Arthritis Breakdown
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Oligoarticular JIA (50-60%)
Polyarticular JIA (30-35%)
Systemic JIA (5-10%)
Enthesitis Related Arthritides (5-15%)
• Juvenile Ankylosing Spondylitis
• Reactive Arthritis
• IBD associated arthritis
• Juvenile Psoriatic Arthritis
ILAR (International League of
Associations for Rheumatology)
Classification of JIA
Case History #1
2 yo girl presents with a 4 week history of limping.
Mom notices she wants to be carried out of bed
in morning and cannot fully straighten her right
leg. But by the afternoon she is running around.
ROS: No fevers. No rash. No recent illnesses.
PE: normal vitals, right leg with swelling, limitation
of motion and pain on passive motion and right
ankle with swelling
Labs: Normal CBC, ESR 25, ANA positive
Oligoarticular JIA
• Fewer than 5 joints
• Tends to affect larger joints and lower extremities
• Asymmetric joint involvement
• Peak onset between 2-4 years
• One knee is the most common presentation (50%)
• Highest risk of uveitis among the JIA types
• Girls:Boys - 3:1
• Labs may be normal or mild elevation of acute
phase reactants
• ANA positive (<1:640) in 65-85%
• Rheumatoid Factor usually negative
Uveitis
• Up to 21% of
oligoarticular and 10%
of polyarticular patients
• ANA positive JIA
patients are at higher
risk of uveitis
• Highest risk of uveitis:
ANA+ young girls with
oligoarticular disease
• Usually asymptomatic
• Can occur anytime
Uveitis - Complications
• cataracts
• glaucoma
• visual impairment,
including blindness
• posterior synechiae
• band keratopathy
Case History #2
14 yo girl presents with hand and feet pain over 6
weeks. It is difficult for her to write in school and
run during her basketball practices. She feels that
her hands are more stiff in the mornings.
ROS: Fatigue. Occasional low grade fevers. No rash.
No recent illnesses
PE: normal vitals, MSK – bilateral wrists, MCPs,
PIPs, knees, ankles, and feet swollen and tender
to palpation
Labs: WBC 7K, Hgb 12, Plt 500 K, ESR 51, ANA
negative, RF positive, anti CCP antibody positive
Polyarticular JIA
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5 or more joints involved
Age of onset: biphasic
Girls:Boys – up to 5:1
Usually symmetric joints
involving the large and small
joints
• Can see boutonnière and swan
neck deformities
• Rheumatoid Factor positive
patients onset in late
childhood or adolescence
• rheumatoid nodules (5-10%)
• early erosive disease
• chronic course persisting into
adulthood
• Can see systemic symptoms of
mild fever, fatigue
Poly JIA – Laboratory
• Usually have elevated acute phase reactants
• Rheumatoid Factor (RF)
• Associated with worse prognosis
• Anti Cyclic Citrullinated Peptide antibodies (anti-CCP
antibodies)
• up to 75% of children with RF+ poly JIA have positive anti CCP
antibodies
Case History #3
• 10 yo boy presents with 2 weeks of high spiking fevers
(>103), rash, joint pains, and body aches. When the fever
is down, he feels fine.
• ROS: positive for chest pain, headaches, sore throat
• PE: T 38.0 HR 120 BP 120/75
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HEENT- cervical LAD
Skin- salmon colored evanescent rash on arms/legs
Lungs- crackles at bases
MSK – bilateral shoulders, elbows, wrist, hips, and knees swollen
and painful on movement
• Labs: Infectious evaluation negative; PPD neg; ESR 105
WBC 25K Hgb 10 Plt 800K AST 60 ALT 75 ANA negative
• Radiology: CXR shows bilateral pleural effusions
Keobner
phenomenom
Systemic Juvenile Idiopathic
Arthritis (SJIA)
• Girls:Boys - 1:1
• Peak age of onset is variable
• Characteristic features: fever 39°C or higher for >2 weeks and
salmon colored rash
• Arthritis joint involvement variable or may be just arthralgias
• Child looks ill during fever, but well when afebrile
• Other features include carditis, pleuritis, pneumonitis, CNS, anemia,
DIC, “macrophage activation syndrome”
• Rash in 80% of patients
• Labs: Elevated ESR, CRP, WBC, Platelets, Ferritin, d-dimers, AST, and ALT.
Anemia, low Albumin, ANA and RF negative
• Uveitis is rare
• Prognosis guarded
SJIA Quotidian Fever Curve
Fever occurs on a once to twice daily basis, usually during the same time of day each
day, spiking to approximately the same temperature each time. The temperature will
often become hypothermic after a fever, prior to return to a normal temperature.
Macrophage Activation
Syndrome
• Concern for Macrophage Activation Syndrome (MAS)
(consumptive process) AKA secondary HLH
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High risk in patients with Systemic JIA
Presents with hemorrhagic syndrome, HSM, LAD, fever
See drop in ESR, Platelets, WBC
Coagulopathy – increased PT/PTT, ↑LFTs, elevated d-dimers,
↓fibrinogen, DIC consumption like picture but also due to liver
dysfunction
• High triglycerides, LDH, FERRITIN (usu > 10,000)
• Mental status changes and seizures
• Can be life threatening
Also reported with: Polyarticular JIA, SLE, MCTD, JDM, Kawasaki’s,
sarcoid
Case History #4
• 16 yo boy presents with lower back pain and
bilateral heel pain that affects his basketball
playing. He is also complaining of a red painful
eye
• ROS: positive for fatigue, some weight loss
• PE: normal vitals
• HEENT – erythematous conjunctiva and sclera
• MSK – bilateral ankle swelling, heel tenderness, SI joint
pain on palpation, and decreased lumbar flexion
• Labs: CBC normal ESR 50 ANA negative, RF
negative, HLA-B27 positive
Enthesitis Related Arthritis
(ERA)/Seronegative Enthesitis Arthritis
(SEA)/ Spondyloarthropathies
• Refers to a group of rheumatic diseases that includes:
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Joints of axial skeleton
Entheses
Psoriasis
Inflammatory bowel disease
Infections
*Can have all or only some of the characteristics
ERA/ Spondyloarthropathy
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HLA- B27 associated
RF and ANA negative
Adult form: inflammatory low back pain
Juvenile form:
• ≤16 years old onset
• Asymmetric arthritis, usually lower extremities
• Peripheral enthesitis
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Age of onset – late childhood and adolescence
Boys>Girls
Arthritis – Axial joint involvement
Enthesitis
Iritis – red painful photophobic eye, usually unilateral
Enthesial evaluation
Iritis: Acute onset of a red,
often painful eye
Case History #5
• 4 yo girl presents with a 3 week history of a swollen toe. No history
of preceding trauma.
• ROS: benign
• PE: normal vitals
• Skin – dry scaly patches on hands and abnormal nails
• MSK – swollen left 3rd toe
• Labs: CBC normal, ESR 30, ANA positive
Juvenile Psoriatic Arthritis
• Psoriasis affects 1-3% of general population and 20-30% of
those patients have associated arthritis
• Preceded, accompanied, or followed by psoriasis
• Half don’t manifest psoriasis until after joint presentation
• Age of onset for arthritis 7 to 11 years, with psoriasis between
9 to 13 years – preschool years and middle to late childhood
• Slightly more common in girls; M:F of 1:2.5.
• Eye
• Asymptomatic uveitis -15-20% and assoc with +ANA
• Symptomatic uveitis - rare in kids and assoc with +HLA-B27
• Arthritis
• Dactylitis - implies underlying tendinitis (aka sausage digit)
• Can be aggressive and damaging
• Scattered, asymmetric oligoarthritis of large and small joints, most
commonly knee, finger (but DIP is rare), toes.
• Pitting of the nails seen in 75% of children with disease.
Juvenile Arthritis Therapy
• Start with nonsteroidal antiinflammatory drugs (NSAIDS)
• If significant synovitis involving multiple joints persists for 3-6
months, or radiologic evidence of destructive disease is
present consider initiation of DMARD (disease modifying
antirheumatic drug). Methotrexate and sulfasalazine are most
commonly used DMARDs.
• If significant synovitis persists despite DMARD consider adding
biologic agent (TNF inhibitors, Tocilizumab, Abatacept).
• Steroids - Never proven to be disease modifying
• Moderate to high doses used for systemic JIA and severe
uveitis (>1 mg/kg/day)
• Low doses for polyarticular JIA and ankylosing spondylitis
(5-15 mg/day)
• Intraarticular steroid injections
Juvenile Arthritis Prognosis
• Oligoarticular JIA - Overall better outcome with less damage
• many will go into remission but subset of children will develop a
polyarticular course
• 20-30% will get uveitis with the sequelae of blindness if unrecognized
and untreated
• Polyarticular JIA – Guarded outcome with potential damage
• prognosis worse if RF+, small joint or hip involvement, or erosive
disease
• patients who had not gone into remission by age 16 are likely to have
chronic course
• Systemic JIA – Guarded outcome
• 50% will recover without problems
• Significant morbidity and mortality can occur with macrophage
activation syndrome
Important Points to Remember
• Arthritis in children can be a symptom of a more systemic process
(non rheumatologic and rheumatologic) OR a disease entity itself
• ANA is only helpful for helping to determine uveitis risk
• Rheumatoid Factor (RF) ONLY important in older children with > 4
joint involvement
• Don’t forget the eyes
• Toe swelling: think psoriatic arthritis
• One knee swelling: think Lyme arthritis
• Any child < 5 years of age with hip or back pain: be cautious!
• JIA pain is more insidious and less acute
• Very rare to have musculoskeletal sprain resulting in acute swelling
in children < 3 years of age
• Xrays are important to rule out fracture or malignancies; not
diagnostic of arthritis