Subacute nonsuppurative cholangitis (cholangitis lenta) in pediatric liver transplant patients

BACKGROUND: Subacute nonsuppurative cholangitis (cholangitis lenta) is an uncommon yet important histological finding in liver biopsies from pediatric liver transplant recipients. The histopathological features include proliferation of bile ductules at the edges of portal tracts, inspissated bile within dilated bile ductules, absence of acute inflammation within ducts or ductules, and normal structure of interlobular bile ducts. OBJECTIVE: To describe the histopathology and clinical outcomes of pediatric liver transplant recipients with subacute nonsuppurative cholangitis. MATERIALS AND METHODS: This is a retrospective analysis involving review of medical records and analysis of liver-biopsy specimens by a pathologist blinded to clinical diagnosis. We identified 9 pediatric patients meeting the criteria for nonsuppurative cholangitis. These patients were compared with a control group of patients with biliary obstruction. RESULTS: Liver histopathology clearly distinguishes subacute nonsuppurative cholangitis from biliary obstruction and other causes of posttransplant cholestasis. Clinical biochemistry cannot reliably distinguish between the 2, although statistically significant differences existed in levels of serum total and direct bilirubin, alanine aminotransferase, and alkaline phosphatase. The rate of proven bacterial or fungal infection in the study group was 100% in contrast to a rate of 54.5% in the control group (P < .05). Graft and patient survival were similarly poor. CONCLUSIONS: Finding subacute nonsuppurative cholangitis suggests the presence of severe local or systemic infection in liver-transplant recipients, and its recognition is important for clinical management.