Refractory Kawasaki disease in neonates with giant coronary aneurysms, intestinal ischemia due to vasculitis and generalized exfoliative dermatitis: a systematic review
DOI:
https://doi.org/10.70577/cekch472Keywords:
Kawasaki disease, refractory, neonate, giant coronary aneurysm, intestinal ischemia, vasculitis, exfoliative dermatitisAbstract
Introduction: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology affecting infants and young children, being extremely rare in neonates and particularly uncommon in its afebrile form (Shen et al., 2024). Refractory KD, defined by persistent or recurrent fever 36 hours after completing intravenous immunoglobulin (IVIG) treatment, affects 15-20% of patients and is associated with high risk of giant coronary aneurysms (Tsoi et al., 2025). Giant coronary aneurysms (Z-score ≥ 10) can complicate with thrombosis, myocardial ischemia, and sudden death (Shrivastava et al., 2026). Mesenteric vasculitis can cause ischemic small bowel strictures, an exceptional complication documented in isolated cases (Beiler et al., 2001; Murphy et al., 1987). Generalized exfoliative dermatitis can present as an atypical manifestation of KD or as a differential diagnosis (Mohammadsaeed & Saeed, 2014). Objective: To synthesize the available evidence on refractory KD in neonates with giant coronary aneurysms, intestinal ischemia due to vasculitis, and generalized exfoliative dermatitis. Methodology: Systematic review following PRISMA 2020 guidelines (Page et al., 2021). A systematic search was conducted in PubMed, LILACS, SciELO and Cochrane for studies published between 1980 and 2026 on refractory KD, giant coronary aneurysms, ischemic gastrointestinal complications, and exfoliative dermatitis. Results: Eight relevant studies were identified. KD in infants under 3 months represents 1.67% of cases and presents higher risk of giant aneurysms (Shrivastava et al., 2026). Infants under 6 months have higher incidence of incomplete KD and diagnostic delay (Shrivastava et al., 2026; Shen et al., 2024). Options for refractory KD include second IVIG dose, methylprednisolone (30 mg/kg/dose), and infliximab (5 mg/kg) (Tsoi et al., 2025). Intestinal ischemia due to mesenteric vasculitis has been documented in cases of incomplete KD, with jejunal stricture and obstruction (Beiler et al., 2001; Murphy et al., 1987). Generalized exfoliative dermatitis can be a manifestation of KD or drug-induced (Mohammadsaeed & Saeed, 2014). Conclusion: Refractory KD in neonates is an exceptional but potentially devastating entity, characterized by rapid progression to giant coronary aneurysms, thrombotic complications, and multisystemic ischemia. Early diagnosis and aggressive treatment with multiple therapeutic lines are essential to reduce associated morbidity and mortality.
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Copyright (c) 2026 Eileen Del Carmen Maurad Farfán, Eduardo Enrique Viteri Parraga, Jennifer Gabriela Monar Villavicencio, Arleth Bianka Sánchez Pacheco, José Luis Ordóñez Galiano, Lizbeth Alexandra Angamarca Jara (Autor/a)
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