The association of sclerosing cholangitis, retroperitoneal fibrosis, and Riedel's thyroiditis has been reported twice before,'2 and on both occasions the authors successfully used steroids to control the fibrotic process. A further case of fibrosis with this triad of organ involvement in which a combination of surgery and steroid treatment has arrested disease progression is described. This sug...

A 9-year-old-girl presented with clinical features of autoimmune hepatitis and associated signs of cholestasis in the form of itching and elevated levels of serum alkaline phosphatase. There was histologic evidence of bile duct injury. Hence a clinical diagnosis of "overlap syndrome" of autoimmune hepatitis with primary sclerosing cholangitis was considered.

Deciding who should receive a liver transplant (LT) depends on both urgency and utility. Most survival scores are validated through discriminative tests, which compare predicted outcomes between patients. Assessing post-transplant survival utility is not discriminate, but should be "calibrated" to be effective. There are currently no such calibrated models. We developed and validated a novel ca...

Purpose To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis. Case Report A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determine...

Autoimmune liver disorders in childhood include autoimmune hepatitis and autoimmune sclerosing cholangitis. These inflammatory liver disorders are characterised histologically by interface hepatitis, biochemically by elevated transaminase levels and serologically by autoantibodies and increased levels of immunoglobulin G. Autoimmune hepatitis is particularly aggressive in children and progresse...

Lipoprotein X (LpX) is an abnormal lipoprotein found in conditions such as lecithin:cholesterol acyltransferase deficiency and cholestatic states (e.g., primary biliary cirrhosis and primary sclerosing cholangitis). Management of severe hypercholesterolemia due to LpX with drugs and physical removal methods is not well established in the literature. A case is discussed of a 51-year-old woman wh...

BACKGROUND Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur. CASE REPORT Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and ther...

IgG4-related sclerosing cholangitis (IgG4-SC) is a characteristic type of sclerosing cholangitis which may be regarded as a biliary lesion of IgG4 related disease [1]. The disease name of IgG4-SC is now well known among gastroenterologists, however, there is a confusing problem about its diagnosis. That is, how to deal with intrapancreatic biliary stricture is very difficult. It is known that I...

Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise ...

Disorders of the hepatobiliary system are relatively common extraintestinal manifestations of inflammatory bowel disease (IBD). These disorders are sometimes due to a shared pathogenesis with IBD as seen in primary sclerosing cholangitis (PSC) and small-duct primary sclerosing cholangitis (small-duct PSC). There are also hepatobiliary manifestations such as cholelithiasis and portal vein thromb...