Rheumatic and Vasculitic Disorders

Question 1

Kawasaki Disease: defined

80% < 5 yo

Answer 1

• Acute vasculitis of medium-sized arteries

- Leading cause of acquired heart disease in U.S., Japan

Question 2

Kawasaki diagnosis:

Answer 2

Fever for at least 5 days, plus 4 of the following:

1) bilateral bulbar conjunctivitis with out exudate
2) intraoral erythema, strawberry tongue, dry/cracked lips
3) nonvesicular rash
4) nonsupporative cervical lymphadenitis
5) erythema/swelling of hands/feet, desquamation of fingertips 1-3 weeks after onset

Question 3

Kawasaki - testing
Labs:
Cardiac finding:

Answer 3

Labs: Increased ESR, CRP @ 4-8 weeks
Platelets increased @ 2-3 weeks (> 1 million)
Cardiac: early myocarditis, pericarditis
cardiac artery aneurysms in 2nd/3rd week

Question 4

Kawasaki: Tx

-steroids show no benefit

Answer 4

1st: IVIG and high-dose aspirin asap, based on clinical
2nd: 2D echo & EKG - get baseline at diagnosis;
- repeat @ 2-3 weeks and at 6-8 weeks
3rd: Add anticoagulant (warfarin) if high risk (high platelets)

Question 5

Kawasaki: prognosis

Answer 5

• Only IVIG reduces incidence of cardiovascular complications
• 1-2% mortality due to coronary artery thrombosis secondary to coronary artery aneurysms

Question 6

HSP: Henoch Shonlein Purpura (leukocytoclastic vasculitis)

- clinical presentation dx

Answer 6

Clinical dx: 2-8 yrs old with -
maculopapular rash (palpable purpura) on legs/buttocks + fever + abdominal pain + viral URI

Question 7

HSP: defined

Answer 7

IgA mediated vasculitis of small vessels (IgA & C3 deposition in skin, kidneys, GIT)

Question 8

HSP: associations

Answer 8

Intussusception
Arthritis
Glomerulonephritis/nephrosis (25%)

Question 9

HSP: tests

- clinical diagnosis

Answer 9

Increased platelets, WBCs, ESR, anemia
Increased IgA, IgM
Anticardiolipin abs or antiphospholipid abs
Urine: WBCs, RBCs, casts, albumin
Definitive: skin biopsy (IgA deposition)

Question 10

HSP: tx

Answer 10

Supportive
-most cases resolve spontaneously
-intestinal/renal complications: steroids
- thrombotic events: aspirin
(+) anticardiolipin or antiphospholipid abs: aspirin