Colangite esclerosante primária: uma revisão narrativa

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of the bile ducts, leading to multifocal strictures, impaired bile flow, and potential progression to cirrhosis and cholangiocarcinoma. Its etiology remains unclear, involving a complex interplay of genetic susceptibility, immune dysregulation, and environmental factors. The strong association with inflammatory bowel disease, particularly ulcerative colitis, suggests that alterations in the intestinal barrier and microbiota may contribute to immunological stimuli affecting cholangiocytes and promoting periductal inflammation. PSC often follows an insidious course, with many patients diagnosed after laboratory evidence of cholestasis, especially elevated alkaline phosphatase; when present, symptoms include fatigue, pruritus, abdominal discomfort, and jaundice. Magnetic resonance cholangiography is the diagnostic modality of choice, while liver biopsy is reserved for selected cases, such as suspected small-duct disease. Despite advances in understanding PSC pathophysiology and in developing investigational therapies, no pharmacologic treatment has yet demonstrated consistent modification of disease progression, and liver transplantation remains the only definitive option for advanced stages. Continued investigation into molecular pathways and robust biomarkers is essential to guide future therapeutic development.