Pediatric Rheumatology

Question 1

[Diagnosis]

Conjunctivitis
Rash
Adenopathy
Strawberry tongue an other oropharyngeal changes
Hand changes (brawny edema, induration)

Answer 1

Kawasaki Disease

Mucocutaneous Lymph Node Syndrome

Question 2

What cardiac finding is the most common characteristic finding of kawasaki disease?

Answer 2

Aneurysm of the major coronary arteries

Question 3

[Clinical Phases of Kawasaki Disease]

Fever and other acute signs of illness, last 1-2 weeks

Answer 3

Acute Febrile Phase

Question 4

[Clinical Phases of Kawasaki Disease]

begins when fever and other acute signs have abated; associated with desquamation, thrombocytosis, development of coronary aneurysm

Answer 4

Subacute phase

Question 5

[Clinical Phases of Kawasaki Disease]

highest risk of sudden death in those who have developed aneurysms; lasts until the 4th week

Answer 5

Subacute phase

Question 6

[Clinical Phases of Kawasaki Disease]

begins when all the clinical signs have disappeared and continues until ESR and CRP return to normal about 6 to 8 weeks after the onset

Answer 6

Convalescent phase

Question 7

[Treatment of Kawasaki Disease]

What is the treatment for acute phase

Answer 7

1. IVIG 2g/kg over 10-12hours

2. Aspirin 80-100 mg/kg/day divided every 6 hours orally until patient is afebrile for at least 48 hours

Question 8

[Treatment of Kawasaki Disease]

Treatment for convalescent stage

Answer 8

1. Aspirin 3-5 mg/kg OD until 6-8 weeks after illness onset

Question 9

[Treatment of Kawasaki Disease]

Long term therapy for patients with coronary abnormalities

Answer 9

1. Aspirin 3-5mg/kg OD PO

2. Clopidogrel 1mg/kg/day (max: 75 mg/day)

Question 10

What is the most common pediatric inflammatory myopathies

Answer 10

Juvenile dermatomyositis

Question 11

____ refers to alligator skin appearance over the metacarpal and proximal IPJ; may be hypertrophic and reddish pink

Answer 11

Gottron papules

Question 12

____ refers to periorbital violaceous erythema that may cross over the bridge of the nose

Answer 12

Heliotrope rash

Question 13

___ refers to use on hands on thighs to stand from a sitting position

Answer 13

Gower sign

Question 14

What is the diagnostic critera for Juvenile Dermatomyositis?

Answer 14

1. Classic Rash (Heliotrope, Gottron) plus 3 of the following
2. Weakness
3. Elevated CK, AST, ALT, LDH, Aldolase
4. EMG changes
5. Muscle biopsy

Question 15

What is the PE finding on the nail for patients with Juvenile Dermatomyositis?

Answer 15

periungual avascularity with capillary dropout and vessel dilatation with terminal bush formation

Question 16

What is the most commonly elevated laboratory parameter in patients with Juvenile Dermatomyositis?

Answer 16

ALT

80% are ANA(+)

Question 17

What antibodies identify a small, distinct group with a protracted disease course with pulmonary and cardiac involvement in patients with juvenile dermatomyositis?

Answer 17

Antibodies to Pm/Scl

Question 18

Drug used for Juvenile Dermatomyositis that decreases the length of treatment with steroids?

Answer 18

Methotrexate

Question 19

What is the most common cause of non-thrombocytopenic purpura in children?

Answer 19

HSP

Question 20

____ IgA-mediated vasculitis of the small vessels (derma capillaries and post capillary venules

Answer 20

HSP

crops of palpable purpura in the dependent parts

Question 21

What are the lab findings in HSP?

Answer 21

1. High ESR
2. Anemia
3. Increased IgA/IgM
4. (-)ANA, (-)ANCA

Question 22

[Juvenile Idiopathic Arthritis]

<4 joints affected
<6 months

Answer 22

JIA, persistent oligo arthritis

Question 23

[Juvenile Idiopathic Arthritis]

> 4 joints affected
6 months

Answer 23

JIA, extended oligoarthritis

Question 24

What is the criteria for diagnosis of SLE?

Answer 24

4 of 11

1. Serositis
2. Oral ulcers
3. Arthritis
4. Photosensitivity
5. Blood changes
6. Renal disorder
7. ANA abnormal titer
8. Immunological changes
9. Neurological signs
10. Malar rash
11. Discoid rash

Question 25

What is the best screening tool for SLE?

Answer 25

ANA

Question 26

What antibody is more specific for lupus and reflects the degree of disease activity

Answer 26

anti-dsDNA

Question 27

Aside from Anti-dsDNA, what other antibodies is specific for SLE

Answer 27

anti-Smith