Rheumatology 101 A Pediatricians Guide Pediatric Staff and Alumni - - PowerPoint PPT Presentation

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Rheumatology 101 A Pediatricians Guide Pediatric Staff and Alumni - - PowerPoint PPT Presentation

cham.org Rheumatology 101 A Pediatricians Guide Pediatric Staff and Alumni Day 2016 Dawn M. Wahezi, Yonit Sterba, Tamar Rubinstein cham.org Disclosures None cham.org Pick a Group Group 1 A child with a limp Group 2 ANA


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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!