Recurrence risk

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Recurrence Rates - The KD Encore

  • Recurrence vs. Recrudescence

    • Recurrence: A new, distinct episode of Kawasaki Disease (KD) after complete recovery.
    • Recrudescence: Relapse of fever and symptoms within days of initial IVIG, indicating treatment resistance.
  • Incidence & Timeline

    • Overall rate is low: ~1-3%.
    • Most recurrences (>50%) happen within 2 years of the initial episode.
    • Risk may be higher in patients who required a second dose of IVIG.

High-Yield Fact: Patients who develop coronary artery abnormalities (CAA) during their first KD episode are at a significantly higher risk for recurrence compared to those without CAA.

Risk Factors - Predicting a Relapse

  • Host-Related Factors:

    • Male sex
    • Age < 1 year at onset
    • Genetic predisposition (e.g., ITPKC polymorphisms)
  • Treatment & Disease Course:

    • Delayed IVIG therapy (> 10 days of fever)
    • Resistance to initial IVIG treatment
    • Severe initial inflammation (↑ CRP, ↑ ESR)
    • Presence of coronary artery lesions (CALs) during the first episode

⭐ Patients resistant to initial IVIG therapy have a significantly higher risk of developing coronary artery aneurysms and subsequent recurrence.

Diagnosis & Features - Spotting the Sequel

  • High Index of Suspicion: Essential for any patient with a history of Kawasaki Disease (KD) presenting with unexplained fever.

  • AHA Criteria for Recurrence:

    • Unexplained fever reappears and lasts ≥48 hours.
    • Accompanied by ≥3 of the original principal clinical criteria.
    • If fever is present with <3 criteria, evaluate for incomplete recurrent KD.
  • Role of Echocardiography:

    • Absolutely critical at the time of suspected recurrence.
    • Aims to detect new or worsening coronary artery abnormalities (CAA).

⭐ Recurrence risk is highest within the first 2 years of the initial illness and is more common in patients who had cardiac complications initially.

Management - The Retreatment Playbook

  • Standard First-Line: IVIG 2 g/kg as a single infusion + high-dose aspirin.
  • IVIG-Resistant KD: Persistent or recrudescent fever ≥36 hours after initial IVIG.
  • Aspirin Regimen:
    • Anti-inflammatory: 80-100 mg/kg/day until afebrile for 48-72 hrs.
    • Anti-platelet: Taper to 3-5 mg/kg/day for 6-8 weeks (or longer if coronary abnormalities).

⭐ Infliximab (5-10 mg/kg), a TNF-α inhibitor, is a key second-line agent, especially effective in refractory cases and for rapid control of inflammation.

High‑Yield Points - ⚡ Biggest Takeaways

  • Recurrence of Kawasaki Disease (KD) is uncommon (approx. 1-3%), with most cases occurring within 2 years of the initial episode.
  • Major risk factors for recurrence include male sex, age <1 year, and significant cardiac involvement during the first illness.
  • Recurrent episodes are often milder and carry a lower risk of developing new coronary artery aneurysms.
  • Management of a recurrent episode is identical to the primary one: high-dose IVIG and aspirin.

Practice Questions: Recurrence risk

Test your understanding with these related questions

A 4-year-old boy is brought to the emergency department by his parents after 10 days of fever, varying from 38.0–40.0°C (100.4–104.0°F). On physical examination, the child is ill-looking with an extensive rash over his trunk with patchy desquamation. His hands are swollen, and he also shows signs of a bilateral conjunctivitis. The laboratory test results are as follows: Hemoglobin 12.9 g/dL Hematocrit 37.7% Mean corpuscular volume 82.2 μm3 Leukocyte count 10,500/mm3 Neutrophils 65% Lymphocytes 30% Monocytes 5% Platelet count 290,000/mm3 Erythrocyte sedimentation rate (ESR) 35 mm/h What is the next best step in the management of this patient’s condition?

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Flashcards: Recurrence risk

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What vasculitis is treated with aspirin? _____

TAP TO REVEAL ANSWER

What vasculitis is treated with aspirin? _____

Kawasaki disease

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