Coronary artery aneurysms

Kawasaki Disease - The Fiery Vasculitis

• Acute febrile illness causing medium-vessel vasculitis, primarily in children < 5 years.
• Diagnosis: Fever for ≥ 5 days plus ≥ 4 of 5 clinical criteria.
• 📌 Mnemonic: CRASH & Burn
  • Conjunctivitis (bilateral, non-exudative)
  • Rash (polymorphous)
  • Adenopathy (cervical, >1.5 cm)
  • Strawberry tongue, lip fissures
  • Hand/foot erythema & edema
• Most serious complication: Coronary artery aneurysms.
• Treatment: IV Immunoglobulin (IVIG) + high-dose Aspirin.

⭐ IVIG (2 g/kg) given within 10 days of fever onset significantly reduces the risk of coronary artery aneurysms.

Diagnosis - Cracking the CRASH Code

• Primary Criterion: Fever ≥ 5 days (high-spiking, unresponsive to antibiotics).
• Principal Clinical Criteria (≥ 4 of 5): 📌 CRASH Mnemonic
  • Conjunctivitis: Bilateral, non-exudative, limbic-sparing.
  • Rash: Polymorphous (maculopapular, morbilliform), non-vesicular. Perineal erythema & desquamation is characteristic.
  • Adenopathy: Cervical, usually unilateral, >1.5 cm.
  • Strawberry Tongue & Lip Changes: Erythema, fissuring of lips, oropharyngeal erythema.
  • Hand & Foot Changes: Acute erythema/edema; subacute periungual desquamation.

⭐ In suspected cases not meeting full criteria (Incomplete Kawasaki), persistent unexplained fever with ↑CRP/ESR warrants echocardiography to rule out coronary artery changes.

Coronary Aneurysms - The Heart of the Matter

• Most feared complication, leading to thrombosis, stenosis, & myocardial infarction (MI).
• Risk ↑ with treatment delay >10 days, male sex, age <1 yr.
• Incidence: 15-25% (untreated) vs. <5% (treated with IVIG).
• Diagnosis & monitoring via Echocardiography at baseline, 1-2 weeks, and 4-6 weeks after treatment.
• Classified by z-scores (internal diameter):
  • Small: z ≥2.5 to <5
  • Medium: z ≥5 to <10
  • Giant: z ≥10 or >8 mm diameter.

⭐ Giant aneurysms have the highest risk of thrombosis/stenosis (~50%) and mandate long-term anticoagulation.

Management & Follow-up - Dousing the Flames

• Primary Goal: Prevent Coronary Artery Aneurysms (CAA).
• Acute Phase (within 10 days):
  • IVIG: Single dose of 2 g/kg over 10-12 hrs.
  • Aspirin: High dose (80-100 mg/kg/day) until afebrile for 48-72 hrs.
• Convalescent Phase:
  • Low-dose Aspirin (3-5 mg/kg/day) for 6-8 weeks if no CAA. Lifelong if CAA present.

⭐ In Kawasaki disease, high-dose aspirin is a cornerstone of initial therapy. Its anti-inflammatory effect is prioritized over the risk of Reye's syndrome, especially in febrile children.

High-Yield Points - ⚡ Biggest Takeaways

• Kawasaki Disease is the leading cause of acquired heart disease in children in most developed nations.
• The most feared complication is coronary artery aneurysms (CAA), seen in 15-25% of untreated children.
• Risk is highest in infants <1 year, those with incomplete presentation, and with delayed IVIG treatment.
• Echocardiography is the gold standard for diagnosis and serial monitoring of coronary arteries.
• Giant aneurysms (>8 mm or Z-score ≥10) carry the highest risk of thrombosis and stenosis.