Cholestatic Liver Injury: Care of Patients With Primary Biliary Cholangitis or Primary Sclerosing Cholangitis

Abstract

The most common causes of chronic cholestatic liver disease are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both disease processes are characterized by a destruction of intrahepatic and/or extrahepatic biliary ducts. The etiology is not entirely clear; however, there is an underlying autoimmune component contributing to both disease processes. Although PBC and PSC are often diagnosed and managed in the outpatient setting, in some instances, a patient may have jaundice, fatigue, and pruritus requiring evaluation and determination of the cholestatic cause. Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms. Liver transplant has the potential for improving quality of life, although disease recurrence is a risk.

Mast Cells in Immune-Mediated Cholangitis and Cholangiocarcinoma

“…In general, PBC is associated with autoimmune diseases (lupus, rheumatic and autoimmune hepatitis) and it occurs predominantly in women [31], high rates of patients show increased levels of circulating auto-antibodies including antinuclear (ANA) and antimitochondrial (AMA) [32]. Moreover, to date several identified genetic risk factors for PBC have been found in autoimmune-related loci such as HLA class II genes as well as in non-HLA alleles (e.g., NF-κB, SOCS1, STAT4) [33,34]. In terms of PSC, circulating auto-antibodies are much less frequent and this disease is strongly associated with autoinflammatory diseases (i.e., ulcerative colitis) rather than autoimmune counterparts [35].…”

Shenqi Fuzheng Injection impairs bile duct ligation-induced cholestatic liver injury in vivo

“…Cholestasis liver injury is a condition that is usually caused by primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), among other predisposing conditions [1]. In patients that present with this condition, pharmacological intervention remains the first clinical option to manage this disease.…”