Primary sclerosing cholangitis (PSC) is a rare, long-term disease of the liver caused by inflammation of tubes in the liver called bile ducts. Scarring and narrowing of bile ducts leads to liver damage. Primary sclerosing cholangitis, a hardening of the bile duct by scarring and repeated inflammation.

Context Explanation

Systemic sclerosis (progressive systemic scleroderma), a rare, chronic disease which affects the skin, and in some cases also blood vessels and internal organs. Primary sclerosing cholangitis (PSC) causes your bile ducts to narrow, blocking the flow of bile. This leads to bile backing up into your liver. Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large ducts in the intrahepatic and/or extrahepatic biliary tree (picture 1) [1-3].

Insight Material

Primary sclerosing cholangitis (PSC) is a rare and progressive hepatic disorder characterized by multifocal inflammatory and fibrotic bile duct strictures. The disease has genetic roots and is linked to autoimmunity. The use of a sclerosing agent to induce fibrous obliteration of pathologic blood vessels–eg, hemorrhoids or esophageal varices. See Endoscopic sclerotherapy, Injection sclerotherapy. Primary sclerosing cholangitis remains a challenging disease to diagnose and manage, due to varying presentations, associated disease processes, and lack of targeted treatment.

Final Conclusion

Primary sclerosing cholangitis (PSC) is a chronic, progressive disease of the bile duct system. The bile duct system carries bile from the liver and gallbladder into the first part of the small intestine, called the duodenum. Patients with PSC may not have any symptoms.