Ch 3 Fever & Rash

Question 1

What’s Kawasaki disease?

Answer 1

Acute febrile multisystem vasculitic syndrome

Question 2

Affected population of Kawasaki Disease?

Answer 2

Children < 5

Asians

Question 3

Most common cause of pediatric acquired heart disease?

Answer 3

Kawasaki

Question 4

What cardiac problem does Kawasaki cause?

Answer 4

Coronary artery aneurysm (killer)
Myo/pericarditis
Valvular abnormalities

Question 5

Cause of Kawasaki?

Answer 5

Unknown

Genetic predisposition to triggered by viral infections.

Question 6

Criteria to Dx Kawasaki?

Answer 6

Fever >39 for >= 5 days and 4 out of 5:

1. Bilateral conjunctivitis
2. Chapped lips / strawberry tongue
3. Unilateral cervical lymphadenopathy
4. Red swollen desquamated hands + feet
5. Rash (morbiliform)

Question 7

1st sign of Kawasaki?

Answer 7

Perianal erythema followed by desquamation.

Question 8

What’s incomplete Kawasaki?

Answer 8

Fever >5 days + coronary artery aneurysm on echo with < 4 of criteria.

Question 9

Other systemic symptoms of Kawasaki?

Answer 9

CNS: aseptic meningitis
MSK: arthritis
GI + GU symptoms.

Question 10

Labs in Kawasaki?

Answer 10

High WBC (Neutrophils)
High Platelets 
Low RBC (anemia)

High ESR/CRP

High transaminases
Low albumin

Sterile pyuria

Question 11

DDx Kawasaki?

Answer 11

Viral exanthem 
Scarlet fever 
SSSS
Drug reaction 
Erythema multiforme 
Stills disease 
Periodic fever syndrome

Question 12

Rx of Kawasaki?

Answer 12

IVIG + aspirin (1st line)

Steroids + infliximab if refractory.

Question 13

What are the hereditary periodic fever syndromes?

Answer 13

• Familia Mediterranean fever (FMF)
• Hyper-IgD with periodic fever syndrome (HIDS)
• TNF receptor associated periodic syndrome (TRAPS)
• Cryopyrin-associated periodic syndrome (CAPS)

Question 14

Pattern of inheritance of hereditary periodic fever syndromes?

Answer 14

1. FMF = AR
2. HIDS = AR
3. TRAPS = AD
4. CAPS = AD

Question 15

Gene defective in hereditary periodic syndromes?

Answer 15

1. FMF = MEFV
2. HIDS = MVK
3. TRAPS = TNFRSF1A
4. CAPS = NLRP3

Question 16

Protein defective in hereditary periodic fever syndromes?

Answer 16

1. FMF = pyrin
2. HIDS = Mevalonate kinase
3. TRAPS = TNF receptor-1
4. CAPS = cryopyrin

Question 17

Episode length in hereditary periodic fever syndromes?

Answer 17

1. FMF = 1-4 days
2. HIDS = 3-7 days
3. TRAPS = > 7 days
4. CAPS = <1-3 days

Question 18

Mucocutanous findings in hereditary periodic fever?

Answer 18

1. FMF = Erysipeloid erythema + edema in leg/foot +/- orogenital ulcer.
2. HIDS = Erythematous papules/macules +/- pruritus +/- orogenital ulcer
3. TRAPS = annular red patches/plaques w distal migration on extremity
   Ecchymotic periorbital edema, conjunctivitis
   +/- oral ulcer.
4. CAPS = urticaria papules/plaques (?cold induced) + conjunctivitis > uveitis
   +/- oral ulcers.

Question 19

Joint involvement in hereditary periodic fever syndromes?

Answer 19

1. FMF = monoarthritis
2. HIDS = oligoarthritis
3. TRAPS = migratory > mono arthritis
4. CAPS = arthritis in NOMID.

Question 20

Rx in hereditary periodic fever syndromes?

Answer 20

1. FMF = colchicine + TNF-inhibitors + IL-1 antagonist
2. HIDS = TNF-inhibitors + IL-1 antagonist + simvastatin
3. TRAPS = Steroids + TNF-inhibitor + IL-1 antagonist.
4. CAPS = IL-1 antagonist