Primary Biliary Cholangitis: Disease Pathogenesis and Implications for Established and Novel Therapeutics

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices

“…Typically, signs and symptoms of PBC include fatigue, jaundice, pruritus, elevated ALP, GGT, and total bilirubin with high titers of AMA present and sometimes ANA as well [ 1 ]. Patients also experience signs of portal hypertension such as ascites, splenomegaly, esophageal varices and hepatic encephalopathy, hepatopulmonary syndrome, hepatorenal syndrome [ 2 ]; however, nonvariceal bleeding in the presence of varices is extremely uncommon. Cirrhosis causing portal hypertension can cause abnormally enlarged and thickened submucosal veins known as varices.…”

Colestasis en el adulto: enfoque diagnóstico y terapéutico. Revisión de tema

“…Las causas intrahepáticas son ocasionadas, generalmente, por condiciones inflamatorias y destructivas que suelen denominarse como el síndrome del conducto evanescente (3,5). La colangitis biliar primaria (CBP), anteriormente conocida como cirrosis biliar primaria, es una de las causas más frecuentes de colestasis de origen autoinmune y genera una colangitis granulomatosa linfocítica que involucra a los conductos biliares de pequeño calibre (3,24,25).…”

Primary Biliary Cholangitis in British Columbia First Nations: Clinical features and discovery of novel genetic susceptibility loci

“…PBC predominantly affects women, with a female:male ratio of 10:1 . Ursodeoxycholic acid, shown to reduce progression of disease and the need for liver transplantation, is the standard treatment for PBC while obeticholic acid is another promising drug under study …”

“…3 Ursodeoxycholic acid, shown to reduce progression of disease and the need for liver transplantation, is the standard treatment for PBC while obeticholic acid is another promising drug under study. 4 Primary Biliary Cirrhosis is considered a multifactorial autoimmune disease, and an interplay of genetic factors and environmental triggers is thought to influence pathogenesis. 3,5 The role of genetic factors in PBC is supported by familial clustering, a high disease concordance in monozygotic twins, and increased prevalence of other autoimmune diseases in PBC patients and their family members compared to the general population.…”