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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!