<unk>  is a relatively rare disease with a progressive nature, characterized by the development of fibrosis and strictures within the intrahepatic and extrahepatic bile ducts. The incidence ranges from 0 to 1.3 per 100,000 cases and prevalence from 0 to 16.2 per 100,000 cases. The disease<unk> ly affects young adult males and the mean age at diagnosis is 41 years. The pathophysiology of<unk>  is not fully understood, although it is thought to be the result of a combination of genetic as well as environmental factors. The risk of<unk>  among siblings is estimated to be 9-39 times higher than the general population. The HLA locus on chromosome 6p21 and genes of the IL-2 pathway are associated with increased susceptibility to the development of<unk> . Environmental factors associated with increased risk of<unk>   include exposure to farm animals during childhood and other dietary habits, such as less consumption of coffee and fish than controls. Smoking seems to be a protective factor against the development of<unk> . The majority of patients with<unk>  are asymptomatic and are only diagnosed following abnormal liver function tests. However, those who are symptomatic often present with nonspecific symptoms, such as fever, fatigue, abdominal pain and/or diarrhea, as well as yellowish discoloration of the skin and mucous membranes. The workup in these patients consists of LFTs, serum immunoglobulins, as well as a number of autoantibodies. Patients with<unk>  typically have an elevated level of ALP, amongst other findings. However, laboratory results in many patients may be normal. ERCP was considered the gold standard for diagnosis of<unk> , because it allows visualization of the biliary tree. However, MRCP is now preferred because of its non-invasive nature. Findings on these imaging modalities include alternating areas of strictures and dilatations of the intrahepatic and extra hepatic bile ducts. Liver transplantation is the gold standard for treatment of<unk>  and there is no effective medical treatment. Ursodeoxycholic acid has been studied as a treatment for the disease. However, there is no proof for its benefit and the American College of Gastroenterology does not support its use in patients with<unk> . Unfortunately, there is no screening for<unk>  sclerosis<unk>  and the prognosis is somewhat poor, even with liver transplantation.
Primary sclerosing cholangitis
Primary biliary cirrhosis
Benign intrahepatic cholestasis
Alcoholic hepatitis
Ischemic hepatitis
Autoimmune hepatitis
Progressive familial intrahepatic cholestasis
Secondary Biliary Cirrhosis