Primary sclerosing cholangitis (PSC) is a chronic disease of unknown cause, leading to cholestasis. What are the symptoms of primary sclerosing cholangitis and how is it treated?

Primary sclerotic cholangitis ( Cholangitis Scleroticans Primaria , PSC) is a chronic disease of unknown etiology. It is believed to be caused by an immune mechanism.

The incidence of PSC is approximately 1 to 5 per 100,000 people per year, more often in men than in women, and the peak incidence is between the ages of 30 and 50.

This may be indicated by the association of PSC with ulcerative colitis, the presence of some alleles of the HLA-DR system and antibodies against the cytoplasm of neutrophils with perinuclear localization (p-ANCA). Primary sclerosing cholangitis is characterized by progressive fibrosis and destruction of various sizes of extrahepatic and intrahepatic bile ducts, leading to alternating narrowing and dilatation, and consequently cholestasis.

Primary sclerosing cholangitis: symptoms

The onset of the disease is usually asymptomatic, and its diagnosis is random, based on laboratory tests - in this case, chronically elevated levels of alkaline phosphatase and GGTP. In some patients, sudden onset of symptoms is possible. They are associated with the development of acute cholangitis caused by infection, which is a complication of asymptomatic biliary obstruction. Patients then complain of fever, tenderness in the upper right quadrant of the abdomen, and sometimes also jaundice.

Later in the disease there is a feeling of progressive fatigue, itching of the skin, chronic jaundice, little characteristic epigastric pain or weight loss. The development of liver cirrhosis is the consequence of the progressive bile duct loss as a result of the inflammatory process. In the end-stage phase of the disease, intraepithelial neoplasm of the bile ducts may develop, which precedes the development of bile duct epithelial cancer. The risk of developing this cancer is 10-15%, and the average time from diagnosis to malignancy is 5 years.

It is especially important that uAlmost 3/4 of patients have coexistence of ulcerative colitis, and other accompanying diseases may be retroperitoneal fibrosis, immunodeficiency syndromes and pancreatitis.

PSC: diagnosis and differentiation

In the first place, it is suggested to perform an ultrasound examination of the liver, which is used to differentiate the type of jaundice and determine its anatomical basis. The examination shows dilated and / or unexpanded bile ducts, mainly intrahepatic with thickened walls.

The diagnosis of PSC is made on the basis of the clinical picture as well as imaging and laboratory tests.

Certain diagnosis is possible with endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP), which show alternating strictures and dilations of the biliary tract. Additionally, MRCP can show thickening of the bile duct walls. Both tests enable the differentiation of changes related to the course of obstructive cholangitis - biliary cysts. Most often it is a cyst of the common bile duct or Caroli's disease, i.e. segmental extensions of the bile ducts (the so-called pseudocysts), often filled with bile deposits. Increased activity of GGTP and alkaline phosphatase are characteristic in laboratory tests. Most patients also have ANCA antibodies against the neutrophil cytoplasm, showing perinuclear fluorescence (p-ANCA) or atypical (x-ANCA) fluorescence. Additionally, microscopic examination of the liver biopsy (material collected during the biopsy), which shows fibrosis around the tract biliary, inflammatory infiltration in the portal spaces and biliary proliferation.

PSC: treatment and prognosis

Please note that pharmacological treatment of PSC is usually unsatisfactory. The use of ursodeoxycholic acid helps to improve the clinical condition of the patient and to normalize the laboratory parameters of cholestasis. It also reduces the risk of developing cancer of the biliary epithelium. Antibiotics are used when a biliary tract infection occurs.

Biliary obstruction can sometimes be treated by surgical bypassing anastomoses or endoscopic insertion of stents. However, it should be borne in mind that such procedures may limit the possibility of a liver transplant being successful, which is the only cure. The average survival time of a patient who has not had a liver transplant is about 10-20 years.

Category:

Gastrology