rheumatology 101 library/global navigation...you suspect that this patient may have jia. what are...
TRANSCRIPT
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Rheumatology 101 A Pediatrician’s Guide
Pediatric Staff and Alumni Day 2016
Dawn M. Wahezi, Yonit Sterba, Tamar Rubinstein
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Disclosures
• None
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Pick a Group
• Group 1 – A child with a limp
• Group 2 – ANA – To test or not to test!
• Group 3 – Picture time! Who would you send to a Pediatric Rheumatologist?
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Group 1 A child with a limp
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Group 1 - Case
2 yo white female is brought to your office because her mother says for the past 6 weeks, she has limped
every morning for about 45 minutes. She does not have any current fever or rash, but her mother does
report that she had a “cold” one month ago.
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Question 1
What findings in the history suggest that this is true arthritis? What would you like to know about her exam to help make this
distinction?
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True arthritis
• Arthalgia vs arthritis – Morning preponderance
– Stiffness/limp > 30 minutes
– Improves with activity
• Definitions – Non-bony swelling
OR
– Two of the following:
• Pain
• Warmth
• Limited ROM
• +/- Erythema
• Signs of chronic disease – Atrophy, leg length discrepancy
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Question 2
What is the differential diagnosis for monoarthritis in this child? What additional investigations would you like to do?
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Differential
• Acute onset < 6 weeks:
• Infectious
– Septic
– Lyme
– ARF/PRSA
– Parvovirus
• Trauma
• Malignancy
• Hemophilia
• Chronic > 6 weeks:
• Rheumatologic
– JIA (oligo, PsA, ERA)
– SLE
– Sarcoidosis
– Other
• Infectious
– Tuberculosis
• Malignancy
Initial work-up: CBC, ESR/CRP, Lyme, ASLO, Parvo IgG/IgM
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Question 3
You suspect that this patient may have JIA. What are the subtypes of JIA and which ones do you suspect in this patient?
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Juvenile Idiopathic Arthritis
• Categories: • Oligoarthritis > 50%
– Persistent
– Extended
• Polyarthritis ~20%
– Rheumatoid Factor Negative Rheumatoid Factor Positive
• Systemic Arthritis ~10%
• Enthesitis Related Arthritis (ERA) ~10%
• Psoriatic Arthritis (PsA) ~10%
• Undifferentiated Arthritis
• Definition: • Arthritis of unknown etiology
• In a child < 16 years old
• Persists over 6 weeks
• Must rule out other causes
What percentage of polyarticular JIA patients will have a positive RF?
Only 15%!
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Joint distribution
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Question 4
What major comorbidity is associated with JIA and how does it manifest itself? What are risk factors for this comorbidity?
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Anterior Uveitis
• Acute uveitis
– Erythema, pain and photophobia
– ERA
• Chronic uveitis
– Minimally symptomatic
– Oligo JIA, PsA
• Complications:
– Cataracts
– Glaucoma
– Synechiea
– Band Keratopathy
Risk Factors: ANA+, age < 7, female, early in dx (< 4 years)
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Group 2 ANA – To test or not to test!
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Group 2
What are the 3 major indications for screening a patient with an ANA?
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ANA testing - Indication # 1
• True arthritis > 6 weeks
– ANA determines uveitis risk in JIA
– May be a presentation of SLE
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ANA testing – Indication # 2
• Raynaud phenomenon – Primary vs Secondary
– SLE, JDM, Scleroderma
• Abnormal nailbed capillaroscopy highly suggestive of underlying rheumatologic disease
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Raynaud Phenomenon
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A RASH POINts MD
• Arthritis
• Renal disease
• ANA positive
• Serositis
• Hematologic disorder
• Photosensitivity
• Oral ulcers
• Immunologic disorder
• Neurologic symptoms
• Malar rash
• Discoid rash
ANA testing – Indication # 3
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What percentage of healthy patients can have a positive ANA?
20 to 30%!
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Positive ANA
• Immunoflorescence: reported as a titer and staining pattern
– Serial dilutions of patient’s
serum the higher the titer, the more dilutions needed to eliminate detection of antibody
– Pattern of nuclear immunofluorescence suggests type of antibodies present in patients serum
Homogeneous
Nucleolar
Peripheral
Speckled
anti-dsDNA, anti-histone
anti-Smith, anti-RNP,
anti-Ro (SSA), anti-La (SSB)
anti-dsDNA
anti-centromere
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Group 3 Picture time! Who would you send to a Pediatric
Rheumatologist?
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Who should see a pediatric rheumatologist?
A B
C D
E
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Gottron’s papules
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Who should see a pediatric rheumatologist?
A B
C D
E
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Mucocutaneous Manifestations of SLE
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Who should see a pediatric rheumatologist?
A
B
C D
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Vasculitis
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Who should see a pediatric rheumatologist?
B
C
A
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Neonatal lupus
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Thank
You!