Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the intra – and extrahepatic bile ducts, resulting in biliary cirrhosis.

 Stages of development:

1. Portal hepatitis
2. Fibrosis (replacement of functioning tissue with connective tissue)
3. Necrosis
4. Cirrhosis of the liver 
5. Risk factors for PSC:
6. Age (up to 40 years)
7. Gender (men)
8. The presence of PSC in relatives
9. Bacteria 
10. Viruses

Clinical manifestations:

1. Jaundice
2. Itchy skin
3. Weight loss
4. Asthenic syndrome (increased fatigue, decreased performance)
5. Increased body temperature
6. Abdominal pain

What complications can be observed in primary sclerosing cholangitis?

* Chronic cholestasis. Chronic cholestasis leads to steatorrhea, deficiency of fat-soluble vitamins (A, D, E, and K), osteoporosis, and weight loss.
* Secondary biliary cirrhosis of the liver. As a result of chronic cholestasis in primary sclerosing cholangitis, secondary biliary cirrhosis of the liver can develop, which leads to portal hypertension, which is manifested by ascites and bleeding from varicose veins.
* Cholelithiasis (gallstones in the gallbladder and bile ducts). Cholelithiasis is common in patients with primary sclerosing cholangitis.
* Cholangiocarcinoma (a malignant tumor of the bile ducts). The development of cholangiocarcinoma is noted in 6-30% of patients with primary sclerosing cholangitis (according to autopsy data in 30-40% of patients). The development of cholangiocarcinoma is unpredictable in each case.
* Strictures (narrowing) of the biliary tract. The formation of bile duct strictures is detected in 20% of cases and requires differential diagnosis with cholangiocarcinoma. Strictures lead to increased cholestasis with the appearance of jaundice and itching.
* Colon cancer. The risk of developing colon cancer in the simultaneous presence of primary sclerosing cholangitis and ulcerative colitis is higher than in the presence of ulcerative colitis alone.

Diagnostics:

* Biochemical blood tests - ALT, AST, GGTP, ALP, bilirubin, albumin, prothrombin. 30% of patients have hypergammaglobulinemia, of which 50% have an increase in immunoglobulin M (IgM).
* Serological examination of blood. The presence of antineutrophilic antibodies (ANCA) - in 87% of cases, anti - cardiolipin antibodies (aCL) - in 66% of cases, antinuclear antibodies (ANA) - in 53% of cases is noted.
* Ultrasound examination of the liver and bile ducts.
* Endoscopic retrograde pancreatocholangiography (ERPHG)- the "gold standard" for the diagnosis of primary sclerosing cholangitis.
* Magnetic resonance pancreatocholangiography (MRPHG).
* Liver biopsy.
* Liver fibroelastometry (allows you to assess the stage of fibrosis without a biopsy)

 Important:
PSC is a chronic disease that tends to progress slowly
70% of patients with primary sclerosing cholangitis are men
the average age of sick men is about 40 years
ultimately, PSC always leads to the formation of hepatic insufficiency (functional liver failure)
the average life expectancy of patients with primary sclerosing cholangitis from the onset of the first symptoms to death is about 7-12 years
the only effective method is liver transplantation!
the survival rate after liver transplantation is 93.7% after 1 year, 86.4% after 5 years, and 69.8% after 10 years%
approximately 15-20% of patients who have undergone liver transplantation, primary sclerosing cholangitis develops again.
Often, the first sign that allows you to suspect changes in the liver is deviations in blood tests.