Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes ...

1 Primary sclerosing cholangitis is a chronic cholestatic syndrome affecting both extrahepatic and intrahepatic bile ducts that is frequently progressive, leading to liver cirrhosis, portal hypertension, and eventually to end-stage liver disease. Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease. The prevalence of inflammatory bowel disease (typically ulcerat...

Primary sclerosing cholangitis is a cholestatic condition with unknown etiology and long-standing, progressive course, leading to cirrhosis and requiring orthotropic liver transplant. In approximately 80%, primary sclerosing cholangitis is accompanied by inflammatory bowel disease, and in most cases the recognition of bowel disease precedes the diagnosis of primary sclerosing cholangitis. We de...

Secondary sclerosing cholangitis is a rare condition caused by disorders directly damaging the biliary tree. We present a case of a 34-year-old man with no pre-existing hepatobiliary disease who developed significant cholestasis and subsequent cholangitis while in the intensive care unit for multiorgan failure secondary to H1N1 influenza A (swine flu). After discharge from the intensive care un...

â  background : primary sclerosing cholangitis (psc) is a chronic cholestatic disease that results in segmental stenosis of the bile ducts, cholestasis and fibrosis. during the previous two decades, patients with steroid responsive psc have been presented in a few case reports and studies. this study aims to evaluate and compare igg4 levels in patients with ulcerative colitis (uc), with and wit...

The association between autoimmune pancreatitis and sclerosing cholangitis has attracted considerable attention. In contrast to type 1 (IgG4-related) autoimmune pancreatitis, bile duct involvement is uncommon in type 2 autoimmune pancreatitis, a more benign condition characterized histologically by granulocytic epithelial lesions (GELs). Following our recent report on a child with GEL-positive ...

The aetiology of primary sclerosing cholangitis is unknown, but it is closely associated with ulcerative colitis. Serum anticolon antibodies, crossreacting with portal tracts, have been reported in patients with ulcerative colitis but no studies have been carried out in primary sclerosing cholangitis. The frequency of serum anticolon antibodies and portal tract antibodies have been measured in ...

The presence of perinuclear antibodies against neutrophils (pANCA) has been detected recently in sera of patients with inflammatory bowel disease and primary sclerosing cholangitis. In order to evaluate their clinical significance, sera from 126 patients with inflammatory bowel disease (80 Crohn's disease and 46 ulcerative colitis and 22 patients with primary sclerosing cholangitis were examine...

Immunoglobulin G4 (IgG4)-related disease is a fibroinflammatory systemic disorder with multiorgan involvement. Proximal bile duct involvement results in IgG4-related sclerosing cholangitis, which is characterized by a lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells and fibrosis. Differentiating between cholangiocarcinoma and IgG4-sclerosing cholangitis can present a diagno...

Aims: To review current issues in the diagnosis and treatment of primary sclerosing cholangitis. Methods: search of PubMed using the terms “primary sclerosing cholangitis”, “PSC”, “cholangitis” and “cholestasis”, as well as by review of current guidelines from the major international liver societies. Results: Primary sclerosing cholangitis is still a challenging condition to diagnose and manage...