pulmicort and Cholangitis

Primary biliary cholangitis is a chronic liver disorder characterized by progressive cholestasis that may evolve to liver cirrhosis. While ursodeoxycholic acid is the treatment of choice, around 30% of patients do not respond to this therapy. These patients have a poorer prognosis, hence should be identified early in order to be offered therapy options. Along these lines, improved understanding of the condition's pathophysiology has allowed the development of newer drugs, including obeticholic acid and fibrates. This review offers a perspective on risk stratification and treatment for these patients, from ursodeoxycholic acid to second-line treatments.

Topics: Adult; Age Factors; Alkaline Phosphatase; Biomarkers; Budesonide; Chenodeoxycholic Acid; Cholagogues and Choleretics; Cholangitis; Cholestasis; Disease Progression; Fibric Acids; Glucocorticoids; Humans; Liver Cirrhosis; Liver Transplantation; Middle Aged; Risk Assessment; Risk Factors; Sex Factors; Treatment Failure; Ursodeoxycholic Acid

The name of chronic liver disease: primary biliary cirrhosis, has been changed to: primary biliary cholangitis, primarily because of the stigma associated with the word "cirrhosis", as only a minority of the patients develop cirrhosis. In this review we present data on epidemiology and discuss the current treatments with focus on ursodeoxycholic acid and the newly described effects of the farnesoid receptor agonist obeticholic acid.

Topics: Anti-Inflammatory Agents; Budesonide; Chenodeoxycholic Acid; Cholagogues and Choleretics; Cholangitis; Fibric Acids; Humans; Liver Cirrhosis, Biliary; Liver Transplantation; Receptors, Cytoplasmic and Nuclear; Ursodeoxycholic Acid

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear. Serologically, PBC is characterized by presence of antimitochondrial antibodies, which are present in 90-95 % of patients and are often detectable years before clinical signs appear. Like other complex disorders, PBC is heterogeneous in its presentation, symptomatology, disease progression, and response to therapy. A significant number of patients develop end-stage liver disease and eventually require liver transplantation. Recent studies from large international cohorts have better identified prognostic factors, suggesting a change in patient management based on risk stratification. Therapeutic options are changing. In this review we discuss data on the autoimmune responses and treatment of the disease.

Topics: Anti-Inflammatory Agents; Autoimmune Diseases; Biopsy; Budesonide; Chenodeoxycholic Acid; Cholagogues and Choleretics; Cholangitis; Fibric Acids; Humans; Immunosuppressive Agents; Liver; Prognosis; Ursodeoxycholic Acid