How Jaundice Occurs in Kawasaki Disease
Jaundice in Kawasaki disease results from systemic inflammation affecting the liver and biliary system during the acute febrile phase, manifesting as hepatitis with elevated transaminases and/or cholestasis with gallbladder hydrops. 1
Pathophysiologic Mechanism
The underlying mechanism involves the widespread vasculitis characteristic of Kawasaki disease, which extends beyond coronary arteries to affect multiple organ systems including the hepatobiliary tract. 1
- Hepatic inflammation occurs as part of the systemic inflammatory cascade, leading to hepatocellular injury and elevated liver enzymes 1
- Biliary tract involvement can cause cholestatic jaundice, often accompanied by hydrops (distension) of the gallbladder 1, 2
- The inflammation is driven by the same immune-mediated process causing arteritis throughout medium-sized vessels in the body 1
Clinical Presentation Patterns
Jaundice in Kawasaki disease typically presents in two forms:
- Mild jaundice with modest transaminase elevation is a recognized associated finding in the standard presentation 1
- Severe obstructive jaundice with cholestasis can occur as an atypical, jaundice-predominant presentation that may precede other classic features 3, 4
Associated Gastrointestinal Manifestations
The hepatobiliary involvement is part of broader gastrointestinal system inflammation: 1
- Diarrhea, vomiting, and abdominal pain commonly accompany the jaundice 1
- Gallbladder hydrops occurs in approximately 50% of jaundice-predominant cases 3
- Pancreatitis may also be present 1
- Some patients develop symptoms mimicking acute abdomen 2
Critical Clinical Implications
Children presenting with jaundice-predominant Kawasaki disease face significantly higher risks and require heightened vigilance: 3
- IVIG-refractory disease occurs in 25-33% of jaundice-predominant cases compared to standard presentations 3
- Coronary artery abnormalities develop in 25-58% of these patients 3
- Shock complicates 13-25% of jaundice-predominant cases 3
- Corticosteroid treatment is required more frequently (24-42%) due to IVIG resistance 3
Diagnostic Pitfalls to Avoid
The presence of prominent jaundice and hepatobiliary symptoms can delay diagnosis because: 4, 5
- Fever with jaundice may initially be attributed to infectious hepatitis, metabolic disorders, or biliary obstruction 4
- Classic mucocutaneous features may appear days to weeks after jaundice onset (sometimes not until day 8-13 of fever) 5
- Gastrointestinal symptoms may lead to surgical admission with other Kawasaki features being overlooked 6
- Persistent fever with jaundice of unknown origin should always prompt consideration of Kawasaki disease to prevent delayed treatment and coronary complications 4, 5
Laboratory Findings
When jaundice is present, expect: 1, 3
- Elevated serum transaminases (hepatocellular pattern) 1
- Direct hyperbilirubinemia in cholestatic presentations 7, 3
- Standard Kawasaki disease markers: elevated ESR, CRP, leukocytosis with left shift, hypoalbuminemia 1
- Thrombocytosis typically emerging in the second week 1
Genetic Considerations
Some evidence suggests that underlying Gilbert syndrome polymorphisms may predispose to more severe jaundice when Kawasaki disease develops, though this remains an area requiring further investigation. 7