Clinical characteristics of 13 patients with primary sclerosing cholangitis
Objective To investigate the clinical characteristics of patients with primary sclerosing cholangitis(PSC)to enhance understanding of the disease and reduce misdiagnosis and missed diagnoses.Methods A retrospective analysis was conducted on the clinical characteristics,diagnostic methods,treatment approaches,and prognosis of patients diagnosed with PSC at the Second Affiliated Hospital of Kunming Medical University from January 2013 to November 2022.Results Of the 13 patients,8(66.7％)were male,with a mean age of 46.1 years and a disease duration of 3(1～26.5)months,Large-duct PSC was diagnosed in 9 patients(69.2％).Twekve patients presented with symptoms,while one was asymptomatic due to hepatic insufficiency.Key symptoms included abdominal pain in 9 patients(75.0％),pruritus in 3(25.0％),jaundice in 7(58.3％),and fatigue in 5(41.7％).One patients had concurrent primary biliary cholangitis,two had chronic pancreatitis,and three(23.1％)had ulcerative colitis.Liver biochemical abnormalities,including elevated liver enzymes,hyperbilirubinemia,and hypoproteinemia,were present in all patients.Immunological testing revealed ANA positivity in 60％(6/10)of patients and pANCA positivity in 25％.MRCP findings indicated bile duct wall irregularities,including uneven thickness,rigid courses,partial strictures and dilatations,and a"withered tree-like"appearance in 2 cases.Of the 10 patients who underwent liver biopsy,only one displayed the characteristic"Onion skin-like"fibrosis.All 13 patients were treated with ursodeoxycholic acid;2 also received hormone therapy,and 2 showed limited response to treatment.The median follow-up duration was 41 months(1-86 months),with one patient lost to follow-up and one death due to acute liver failure.Conclusion PSC primarily affects middle-aged men and predominatly involves large-duct disease.Its clinical manifestations are nonspecific,frequently accompanied by ulcerative colitis,and may co-occur with other autoimmune liver diseases.Cholangiography remains the primary diagnostic tool,while treatment with ursodeoxycholic acid can improve clinical outcomes.
万方数据
维普
