Infliximab in IVIG-resistant Kawasaki disease with cholestatic hepatitis: case report

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Frontiers in Medicine Published April 16, 2026 Medically reviewed April 25, 2026 DOI ↗ By Dr. Sofia Müller, MD · Lifespan & Whole-Person Care

Key Takeaway

Consider dissociation between systemic and coronary effects in infliximab use for IVIG-resistant KD.

This case report involves a 2-year-old Han Chinese male with IVIG-resistant Kawasaki disease and cholestatic hepatitis. The patient received infliximab (5 mg/kg) after failing initial high-dose IVIG (2 g/kg) combined with clopidogrel and a second dose of IVIG (1 g/kg) combined with high-dose methylprednisolone pulse therapy. The primary outcome was prevention of coronary artery aneurysm (CAA) progression, with secondary outcomes including fever subsidence, inflammatory indicators (CRP, ESR), and liver function indicators (ALT, AST, bilirubin, total bile acids).

Main results showed fever subsided rapidly within 24 h, and inflammatory and liver function indicators improved significantly. However, coronary artery aneurysms progressed, eventually forming giant CAAs. Echocardiographic follow-up was conducted, but specific durations or numerical improvements in markers were not reported. Safety and tolerability data were not provided.

Key limitations include dissociation between systemic inflammation control and coronary protection, and the critical importance of optimizing the timing of infliximab administration. Practice relevance suggests further randomized controlled trials are needed to clarify optimal timing, dose, and patient selection criteria for infliximab in IVIG-resistant Kawasaki disease. This case underscores the need for careful monitoring in such complex presentations.

Study Details

Study typeRct

EvidenceLevel 2

PublishedApr 2026

View Original Abstract ↓

BackgroundKawasaki disease (KD) is the leading cause of acquired heart disease in children under 5 years of age worldwide. Approximately 15%–20% of KD patients are resistant to intravenous immunoglobulin (IVIG), and these patients have a significantly higher risk of developing coronary artery aneurysms (CAAs), a severe complication leading to long-term cardiovascular morbidity. Cholestatic hepatitis is a rare manifestation of KD, which further increases the difficulty of clinical treatment. This study reports a case of IVIG-resistant KD complicated with cholestatic hepatitis treated with infliximab, and explores the clinical challenges in preventing CAA progression.Case reportA 2-year-old Han Chinese male presented with persistent fever as the initial symptom, followed by typical clinical manifestations of KD and cholestatic hepatitis. Initial treatment with high-dose IVIG (2 g/kg) combined with clopidogrel (1 mg/kg/day) was ineffective. A second dose of IVIG (1 g/kg) combined with high-dose methylprednisolone pulse therapy also failed to control the disease. On the ninth day of illness, salvage therapy with the tumor necrosis factor-alpha (TNF-α) inhibitor infliximab (5 mg/kg) was administered, and the child's fever subsided rapidly within 24 h. Subsequent laboratory examinations showed that inflammatory indicators [C-reactive protein (CRP), erythrocyte sedimentation rate (ESR)] and liver function indicators [alanine aminotransferase (ALT), aspartate aminotransferase (AST), bilirubin, total bile acids (TBA)] improved significantly. However, despite effective inflammation control, echocardiographic follow-up revealed progressive development of CAAs, eventually forming giant CAAs.ConclusionInfliximab can effectively suppress systemic inflammation and improve liver function in patients with IVIG-resistant KD complicated with cholestatic hepatitis. However, the development of giant CAAs in this case underscores the dissociation between systemic inflammation control and coronary protection, highlighting the critical importance of optimizing the timing of infliximab administration. Further randomized controlled trials are needed to clarify the optimal timing, dose, and patient selection criteria for infliximab in the treatment of IVIG-resistant KD.