DefinePK

Abstract

Background: Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive hepatic disorder characterized by intermittent episodes of cholestatic jaundice, pruritus, and fatigue. Despite alarming biochemical derangements, BRIC does not lead to cirrhosis or chronic liver failure. Due to its self-limiting nature and rarity, it is often underdiagnosed, particularly in developing countries.
Aims: To describe the clinical presentation, laboratory features, imaging, histopathological findings, and therapeutic outcomes in five histologically confirmed cases of BRIC and raise awareness of this underrecognized entity.
Methods:A retrospective case series was conducted at a tertiary care centre in Belagavi, India. Five patients diagnosed with BRIC between 2021 to 2024 were studied. Data were obtained from the Department of Gastroenterology and Medical Records, including demographics, symptoms, liver function tests, imaging (USG and MRCP), histopathology, treatment, and outcomes.
Results:All five patients (4 males, 1 female; age range: 14–24 years) presented with recurrent episodes of jaundice and pruritus. Liver function tests consistently showed cholestatic patterns with elevated direct bilirubin and alkaline phosphatase, and relatively preserved transaminases. Viral, autoimmune, and metabolic causes were excluded. Imaging (USG and MRCP) revealed no biliary obstruction or architectural liver damage. Liver biopsy demonstrated canalicular and intrahepatic cholestasis. All patients responded well to supportive management, including ursodeoxycholic acid and cholestyramine, with resolution of symptoms.
Discussion:BRIC, while benign, can cause significant distress due to recurrent jaundice and itching. The episodic nature, lack of imaging findings, and histology-driven diagnosis necessitate a high index of suspicion. In our series, patients responded favourably to conservative management without long-term complications or disease progression.
Conclusion:BRIC should be considered in young patients presenting with episodic cholestatic jaundice and normal imaging findings. Prompt diagnosis through histopathological confirmation avoids unnecessary interventions. Long-term outcomes are excellent with appropriate symptomatic management and patient counselling