board review: pediatric rheumatology kathy haines, md jennifer weiss, md chief of pediatric...
TRANSCRIPT
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Board Review: Pediatric
Rheumatology
Kathy Haines, MDJennifer Weiss, MD
Chief of Pediatric RheumatologyJoseph M. Sanzari Children’s HospitalHackensack University Medical Center
Associate Professor of PediatricsUMDNJ-New Jersey Medical School
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Arthritis
More than 100 causes of arthritis in children
Arthritis is common in rheumatic diseases
But not all rheumatic diseases are associated with arthritis
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Acute Causes of Arthritis Traumatic
Hemarthrosis Infectious
Bacterial (septic arthritis, osteomyelitis, Lyme) Viral (esp rubella, parvo)
Post-Infectious Reactive arthritis Toxic/transient synovitis
Allergy Serum sickness
Acute Inflammatory and Rheumatic Conditions HSP Kawasaki disease Rheumatic fever
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Most Common Causes of Chronic Arthritis in Children
Juvenile Idiopathic Arthritis What we used to call “JRA”
PLUS Juvenile Spondyloarthritis Juvenile Psoriatic Arthritis
Other chronic rheumatic syndromes SLE Dermatomyositis Vasculitis
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Other causes of joint pain:Mimics of arthritis
Mechanical abnormalities Patellofemoral Syndrome Hypermobility Syndromes
Malignancies Endocrine abnormalities Inherited bony dysplasias Chronic pain syndromes Psychogenic causes
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Acute Rheumatic Syndromes
Reactive arthritis Transient/toxic synovitis
Henoch Schonlein PurpuraKawasaki Disease
Acute Rheumatic Fever
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Reactive Arthritis(aka Reiter Syndrome) Arthritis and other extra-articular features following infections Classical triggers:
Enteric (Salmonella, Shigella, Yersinia) Non-gonoccoal urethritis
Other infections commonly cause reactive arthritis Varicella Parvovirus Group A Streptococcus (rheumatic fever and post-strep reactive
arthritis) Many other nonspecific infections
Extra-articular features: Conjunctivitis or uveitis Urethritis Rash (keratoderma blenorrhagicum) Not common in children
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Transient synovitis
Acute hip synovitis in toddlers-early school age Often follows onset of URI Usually unilateral leg (hip) pain May have low grade fever Labs usually normal PE: Pain and limitation of motion of affected hip Self-limited: resolves within a few days to weeks
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Henoch Schonlein Purpura
Common acute vasculitis of childhood Manifestations:
Purpura Usually limited to lower extremities and buttocks
Arthritis Nephritis Abdominal pain
Usually self-limited (resolves after a few weeks) Often post-infectious (especially Strep)
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HSP
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HSP continued Purpuric rash on the LE is almost pathognomonic
(in the absence of infection or coagulopathy) Biopsy:
Leukocytoclastic vasculitis (seen in many other types of vasculitis)
IgA deposits on IF (diagnostic for HSP) Nephritis:
IgA nephropathy Usually manifests as hematuria which resolves Need to check urinalysis frequently (weekly) for a month
and then monthly Some develop chronic renal disease
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HSP Treatment
Symptomatic NSAIDs (joints) Steroids (reserved for significant GI involvement)
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Kawasaki Disease Most common vasculitis in young children Usually 6 years or younger Criteria (5 of 6 must be present for definite dx)
1. High grade fever for more than 5 days
2. Polymorphous rash (MP, scarlatiniform, morbilliform), often starts in the groin and diaper area
3. Non exudative conjunctivitis
4. Cervical lymphadenopathy >1.5 cm (usually unilateral)
5. Mucositis (strawberry tongue, red lips and mouth, vertical cracking of lips)
6. Extremity changes (red palms and soles, edema of hands and feet)
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KD conjunctivitis
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Polymorphous rash of KD
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Typical mucositis of KD
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Unilateral lymphadenopathy
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Acute Extremity Changes of KD
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Late extremity manifestations of KD
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Kawasaki Disease: Labs
Labs reflect highly inflammatory state Elevated WBC, ESR, CRP Thrombocytosis comes later (1 to 2 wks after
onset) Elevated LFTs (transaminases) common Sterile pyuria Aseptic meningitis
No specific diagnostic test
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Kawasaki disease treatment Treatment
IVIG (2gm/KG): best outcome if prior to 10 days of fever Low dose aspirin Other (for refractory cases):
Pulse steroids Anti-TNF therapy
Complications: Coronary artery aneurysms Myocarditis Myocardial infarction
Atypical KD patients that do not fulfill criteria is not uncommon Fever, rash, conjunctivitis most common features
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Acute Rheumatic Fever
Post-Streptococcal rheumatic syndrome Fever (usually low grade) and migratory
polyarthritis 1-2 weeks following Strep infection (which can be subclinical)
Carditis is common but not always present Mitral insufficiency most common
Acute phase reactants (ESR and CRP) always elevated
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ARF Jones Criteria 2 Major criteria, or 1 Major and 2 minor criteria Major
Migratory polyarthritis Carditis (valvulitis, especially mitral) Erythema marginatum Subcutaneous nodules Chorea
Minor Fever Elevated ESR Arthralgias (doesn’t count if has polyarthritis as major)
Prolonged PR interval
Required: Evidence of recent Strep infection
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Uncommon Manifestations of ARF: Nodules and erythema marginatum
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Treatment of Acute Rheumatic Fever
Aspirin or NSAIDs The arthritis will “melt away” Must be taken consistently until ESR normalizes
Steroids reserved for severe carditis Penicillin prophylaxis (until adulthood?)
PO Monthly IM bicillin
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A 6 year old boy has a 24 hr history of fever, malaise, and bruising. He appears ill and his temperature is 103. He has widespread petechiae and palpable purpura on the buttocks and lower extremities. The hgb is 10.5, WBC 22.5, and platelets are 25,000. The most likely diagnosis is:
1 2 3 4 5
13%
30%
23%
10%
23%1. Idiopathic
thrombocytopenic purpura
2. HSP
3. Leukemia
4. Meningococcemia
5. Rocky Mountain spotted fever
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An 8 year old girl has a painful left knee. Two days ago her right ankle was painful and swollen, but today it seems fine. She has a fever to 101, and her resting heart rate is 120. Her knee is swollen and painful, but her ankle is completely normal. She has a Grade 3/6 systolic murmur. The most likely diagnosis is:
1 2 3 4 5
30% 30%
7%
10%
23%1. Dermatomyositis
2. Systemic JIA
3. Acute rheumatic fever
4. Septic arthritis
5. Systemic lupus erythematosus
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A 5 year old boy has been limping for 3 days. He had an URI earlier in the week and there is no hx of trauma. His temp is 99.9, and he is limping. There is pain with flexion and internal rotation of his right hip. The WBC is 4.5 and ESR is 20. The most likely diagnosis is:
1 2 3 4 5
13%
23%
30%
27%
7%
1. Legg Perthes disease
2. Slipped capitol femoral epiphysis
3. Oligoarticular JIA
4. Septic arthritis
5. Transient synovitis
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Chronic Arthritis in Children
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Chronic Arthritis in Children(JRA and JIA)
Pauciarticular JRA / Oligoarticular JIA Polyarticular JRA / Polyarticular JIA
RF positive RF negative
Systemic JRA / Systemic JIA Spondyloarthropathies / Enthesitis related
JIA Psoriatic arthritis / Psoriatic JIA
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JRA/JIA Definition
Must have had arthritis in at least one joint for > 6 weeks
Must be less than 16 years old at onset of symptoms
Must exclude all other conditions No tests are diagnostic
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Pauciarticular JRAOligoarticular JIA Definition
Four or less joints Usually little girls (average
age: 2 yrs) Insidious onset of a swollen
joint, most often the knee Labs usually normal except:
ESR may be mildly elevated
ANA often positive High risk of iritis/uveitis
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Uveitis in JIA
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Bone deformities, contractures& atrophy
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Polyarticular JRAPolyarticular JIA
Definition: 5 or more joints involved No systemic sx RF serologic status is
important in classification RF negative poly JIA
(young girls: mean age 4) RF positive poly JIA
(older pre- to teen aged girls >10 years)
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RF Negative Polyarticular JIA >5 joints, but usually has symmetrical arthritis in many
joints, including small joints RF negative ANA + in 50% or more Elevated ESR, CRP, Ig’s Usually younger girls than RF positive Moderate risk for uveitis
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RF Positive Polyarticular JIA
Positive rheumatoid factor test
Identical to adult Rheumatoid Arthritis
Pre-teen/teen onset most frequent
Similar joint pattern to RF negative polyarthritis
Elevated ESR, CRP Hallmark: rheumatoid
nodules
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Systemic JRA or JIA Fevers
High (>39) Quotidian pattern is common
Rash Non-fixed pink eruption Worsens with fever spike Koebner phenomenon
Arthritis (any number of joints can be affected)
Other features: Generalized lymphadenopathy Hepato- or splenomegaly Serositis (Pleuritis or
pericarditis)
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Quotidian Fever Pattern
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Systemic JIA Laboratory features
High WBC (poly predominant) Anemia common Thrombocytosis (often 5-800,000) Very high ESR and CRP Extremely high ferritin levels (500-10,000+) Negative serologies
Other complications Tamponade Macrophage activation syndrome (can be fatal) Pulmonary hypertension Uveitis is not seen
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Juvenile SpondylarthropathiesEnthesitis related JIA
Arthritis and enthesitis (usually calcaneal) More common in pre-teens and teens Boys > girls
Other features HLA B-27 commonly positive Acute anterior uveitis Sacroiliitis Inflammatory spine pain and limitation of flexion Positive family history: anterior uveitis,
spondylarthropathies, especially ankylosing spondylitis, Reiter syndrome and inflammatory bowel disease
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Spondylarthropathy / Enthesitis related JIA Features
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Juvenile Psoriatic ArthritisPsoriatic JIA
Arthritis and psoriasisOR
Arthritis and a positive family history of psoriasis plus dactylitis nail pitting or
onycholysis
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Psoriatic arthritis features
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Psoriatic arthritis
Can mimic any of the following: Oligoarticular JIA Polyarticular JIA Enthesitis related JIA and ankylosing spondylitis
Dactylitis (isolated swollen digits) is the most specific type of arthritis associated with arthritis
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Dactylitis in psoriatic arthritis
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JIA Treatment in a nutshell Oligoarticular forms of JIA
NSAIDs Intra-articular steroids
Polyarticular forms of JIA NSAIDs Methotrexate Prednisone is not usually needed Anti-TNF biologics
Systemic JIA NSAIDs Steroids for severe systemic symptoms (especially pericarditis) Methotrexate for arthritis Anti-IL1 and IL6 biologics
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A 6 yr old boy has a 4 week hx of fevers to 40 deg C once a day accompanied by a fleeting rash. Physical examination reveals generalized lymphadenopathy. Of the following, the most common other manifestation of this patient’s illness would be:
1 2 3 4 5
20%
30%
17%
23%
10%
1. Marked leukocytosis
2. Positive ANA
3. Positive RF
4. Joint pain
5. Uveitis
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A 2 year old girl presents with a 2 month history of intermittent limping. She cries when she first gets up in the morning and won’t walk, but later on in the day she is much better. She has had no fever. On exam, she is happy and playful until you examine her right knee, which is swollen and warm and has decreased flexion. She is unable to straighten it completely. Her labs are normal but her ANA is positive. Which of the following is most likely to develop in this child:
1 2 3 4 5
13%
27%
17%
10%
33%
1. Psoriasis
2. Lupus
3. Uveitis
4. Rheumatoid nodules
5. Muscle weakness
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Major Rheumatic Conditions
SLE
Dermatomyositis
Scleroderma
Vasculitis
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Childhood Systemic Lupus Erythematosus
10% of pediatric rheumatology patients It is a systemic disease that usually affects
more than one organ Variable presentation
Acute fulminant onset with life-threatening complications
Chronic insidious onset (can be difficult to diagnose)
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Lupus is a systemic disease Rash (Malar rash classic, but many other skin manifestations) Arthritis Cytopenia
Leukopenia and thrombocytopenia common Coombs’ positive hemolytic anemia
Nephritis (proteinuria alone or with hematuria) Can present with nephrotic syndrome or renal insufficiency
CNS manifestations Seizures Psychosis Other less common (transverse myelitis, coma, stroke)
Pulmonary and/or cardiac involvement (especially pleuro-pericarditis)
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Laboratory testing for SLE Basic labs are important:
CBC (may see cytopenias) ESR usually elevated in active lupus Renal and hepatic function (chemscreen) Urinalysis: proteinuria and hematuria
Serologies: ANA (almost always positive, but not specific and can be falsely
positive) Anti-dsDNA (much more specific for lupus, and can be used to
follow disease activity) Complement levels are low in active SLE
C3 and C4 Total complement
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Other Antibody Tests in SLE Anti-ENA
Anti-Sm or Smith (highly specific for SLE) Anti-RNP (associated with mixed connective tissue
disease) Anti-SSA (Ro) and SSB (La)
Associated with Sjogrens as well as lupus Positive in mothers of babies who develop neonatal lupus
Anti-phospholipid antibodies Anti-cardiolipin, anti-phosphatidyl serine, lupus
anticoagulant and beta-2 glycoprotein I Associated with thromboembolic disease (pulmonary
emboli, deep vein thrombosis, frequent miscarriages, arterial thrombosis)
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Treatment of Lupus Prednisone and pulse methylprednisolone
For acute treatment of severe organ involvement (kidneys, CNS, pericarditis)
Hydroxycholoquine (Plaquenil) Arthritis Rash General treatment of lupus
Methotrexate Arthritis
Cyclophosphamide, azathioprine or mycophenolate Significant renal and other organ system involvement
Anti-B cell therapies (Rituximab)
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Neonatal Lupus Babies born to mothers with
lupus or positive anti-SSA/SSB antibodies: Immune cytopenia Rash Congenital heart block
can cause hydrops and fetal death
Congenital heart block highly associated with: Anti-SSA/Ro and/or Anti-
SSB/La May be asymptomatic
but antibody positive
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Juvenile Dermatomyositis
Inflammatory myositis (causing muscle weakness, not usually pain) and specific dermatitis
Triad Diagnostic rash Proximal muscle weakness Elevated muscle enzymes
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Skin Findings of Dermatomyositis
Gottron’s papules Heliotrope rash Extensor rash Nailfold telangiectasias and cuticular
hypertrophy Photosensitive rash (can be mistaken for
lupus) Malar rash Shawl distribution of neck and shoulders
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JDM Gottron’s Papules
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Heliotrope rash
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JDM Nailfold Telangiectasias
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JDM extensor rash
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JDM photosensitive malar rash
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Muscle findings in dermatomyositis
Symmetrical PROXIMAL>>>distal weakness of all four extremities
Muscle enzymes ARE elevated CK, AST, ALT, LDH, Aldolase
Trunk, neck flexors, pharyngeal and respiratory muscles can be involved
EMG and muscle biopsy may not be necessary if classical features present Rash, proximal muscle weakness, elevated enzymes
MRI: edema of muscles consistent with myositis
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JDM complications: ulcerations
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JDM complications: calcinosis
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Gower maneuver in the younger child
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Dermatomyositis Treatment
Steroids Methotrexate Other
Cyclosporine IVIG Cellcept Cyclophosphamide Rituximab
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Scleroderma
Systemic scleroderma Progressive Systemic Sclerosis CREST syndrome (Calcinosis, Raynaud’s,
Esophageal dysmotility, Sclerodactyly, Telangiectasias)
Localized scleroderma Morphea Linear Scleroderma
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Systemic Scleroderma
Severe Raynaud’s phenomenon Diffuse tightening of the skin
Progresses from distal to proximal and truncal Cardiopulmonary disease
Interstitial lung disease Pulmonary hypertension Cor pulmonale
Hypertensive renal crisis
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Serologies in Scleroderma
ANA usually positive Anti-SCL 70
Systemic sclerosis Anti-Centromere
CREST syndrome Anti-RNP
Mixed connective tissue disease often has sclerodermatous features
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Raynaud’s Phenomenon in Scleroderma
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Skin Features of Systemic Sclerosis
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Localized Scleroderma
Not related to systemic sclerosis Two major types:
Morphea Linear Scleroderma
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Morphea
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Linear Scleroderma
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Treatment of Scleroderma
Systemic sclerosis Symptomatic treatment
Raynaud’s (nifedipine, sildenafil) Joints (steroids and methotrexate)
Pulmonary hypertension Anti-fibrotic treatment may prove to be helpful
Localized scleroderma Steroids and methotrexate may be effective for
linear scleroderma or severe morphea
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A 9 year old girl has generalized weakness and a rash. Findings include a malar rash and erythematous papules over her PIP joints and swollen red cuticles. Her proximal strength is 3/5. Of the following, the most appropriate next step in her evaluation is:
1 2 3 4 5
13%
27% 27%27%
7%
1. ANA titer
2. CK concentration
3. EMG
4. MRI of muscle
5. Complement levels
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A male infant was delivered prematurely because of progressive hydrops to a 34 yo woman. He was found to have complete heart block. Of the following, the most useful lab test in confirming the diagnosis of neonatal lupus is:
1 2 3 4 5
23%
10%
23%
17%
27%
1. Antinuclear antibody
2. Anti-Ro (SSA) and anti-La (SSB)
3. Human Lymphocyte Antigens
4. Serum complement level
5. Anti-Smith (Sm) levels
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A 13 year old female has fever, myalgias and joint swelling for a month. She has arthritis, palatal ulcers and small ulcerations on her fingertips. The WBC is 3.5K and platelets are 120,000. Of the following the most helpful test to confirm the diagnosis is:
1 2 3 4 5
33%
27%
10%
13%
17%1. ASO titer
2. C3 level
3. C-reactive protein
4. Anti-dsDNA titer
5. ESR