Vasculitis ( Pediatrics)

Question 1

Henoch-Schonlein purpura clinical picture and diagnosis.

Answer 1

IgA-mediated vasculitis :
■ Palpable purpura (usually lower extremities), joint pain, abdominal pain +/- intussusception, hematuria (IgA nephropathy).

● Usually occurs after viral/streptococcal upper respiratory infection.
● If severe abdominal pain, do US to check for intussusception.

Question 2

Henoch-Schonlein purpura lab findings

Answer 2

Normal platelets/ coagulation (distinquish from ITP).

Normal to increased creatinine.

Hematuria.

+/-RBCs casts and proteinuria.

Question 3

Henoch-Schonlein purpura treatment

Answer 3

● Usually supportive (hydration/NSAIDs).

● If severe GI pain/ renal involvement, use steroids.

● If hypertensive, use ACE-inhibitors.

● Conduct regular urinalysis for the first 6 months after diagnosis to re-evaluate if it becomes chronic IgA nephropathy.

Question 4

Definition of Kawasaki Disease

Answer 4

Multisystem inflammation of medium-sized blood vessels.

Peak incidence between 6-11 months, usually before age 4.

Question 5

Clinical presentation and symptoms of Kawasaki Disease

Answer 5

CRASH and Burn: mnemonic :
● C: Conjunctivitis (bilateral)
● R: Rash. Polymorphic, typically truncal. Later becomes desquamating. Includes palms/soles
● A: Adenopathy (cervical)
● S: Strawberry tongue (oral mucositis)
● H: Hand/feet erythema/edema.
● Burn: Fever > 5 days w/o explanation (typically 39-40 ℃). Usually self-limited fever lasts 12-14 days.

Question 6

Labs of Kawasaki Disease

Answer 6

ESR, CRP, WBC are all really high.

Question 7

Complications of Kawasaki Disease

Answer 7

High risk of coronary artery aneurysm with thrombosis.

Perform repeated echos to monitor.

Give aspirin.

Question 8

Differential Diagnosis of Kawasaki Disease

Answer 8

Scarlet fever
EBV
Drug reaction
Systemic JIA
Sepsis

Question 9

Treatment of Kawasaki Disease

Answer 9

IVIG & aspirin to prevent clot formation.

Decrease inflammation.

Give steroids if refractory to IVIG.