Rheumatology 101 A Pediatrician s Guide Pediatric Staff and Alumni Day 2016 Dawn M. Wahezi, Yonit Sterba, Tamar Rubinstein
Disclosures None
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?
Group 1 A child with a limp
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.
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?
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
Question 2 What is the differential diagnosis for monoarthritis in this child? What additional investigations would you like to do?
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
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?
Juvenile Idiopathic 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%! 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
Joint distribution cham.org
Question 4 What major comorbidity is associated with JIA and how does it manifest itself? What are risk factors for this comorbidity?
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)
Group 2 ANA To test or not to test!
Group 2 What are the 3 major indications for screening a patient with an ANA?
ANA testing - Indication # 1 True arthritis > 6 weeks ANA determines uveitis risk in JIA May be a presentation of SLE
ANA testing Indication # 2 Raynaud phenomenon Primary vs Secondary SLE, JDM, Scleroderma Abnormal nailbed capillaroscopy highly suggestive of underlying rheumatologic disease
Raynaud Phenomenon cham.org
ANA testing Indication # 3 A RASH POINts MD Arthritis Renal disease ANA positive Serositis Hematologic disorder Photosensitivity Oral ulcers Immunologic disorder Neurologic symptoms Malar rash Discoid rash
What percentage of healthy patients can have a positive ANA? 20 to 30%!
Positive ANA Immunoflorescence: reported as a titer and staining pattern Homogeneous Peripheral Serial dilutions of patient s serum the higher the titer, the more dilutions needed to eliminate detection of antibody anti-dsdna, anti-histone anti-dsdna Pattern of nuclear immunofluorescence suggests type of antibodies present in patients serum anti-smith, anti-rnp, anti-ro (SSA), anti-la (SSB) Speckled anti-centromere Nucleolar
Group 3 Picture time! Who would you send to a Pediatric Rheumatologist?
Who should see a pediatric rheumatologist? A B E C D
Gottron s papules cham.org
Who should see a pediatric rheumatologist? A B E C D
Mucocutaneous Manifestations of SLE cham.org
Who should see a pediatric rheumatologist? A B C D
Vasculitis cham.org
Who should see a pediatric rheumatologist? A B C
Neonatal lupus cham.org
Thank You!