What happens in the liver
Immune cells attack the epithelium of the small intrahepatic bile ducts, causing their chronic inflammation and gradual disappearance. The outflow of bile becomes difficult, it accumulates and has a toxic effect on liver cells. The body responds with the proliferation of connective tissue: fibrosis develops, which, over a long period of time, turns into cirrhosis with its complications. At the same time, the absorption of fats and fat-soluble vitamins is impaired, cholesterol levels rise, and bone strength suffers.
- Autoimmune destruction of small bile ducts
- Stagnation of bile inside the liver
- Liver cell damage and fibrosis
- Formation of cirrhosis over a long period of time
- Impaired absorption of fat-soluble vitamins
- Increased cholesterol levels
Symptoms
About a third of patients have no complaints at all at the time of detection. The earliest and most characteristic symptoms are debilitating fatigue that does not go away after rest, and itching of the skin, which intensifies in the evening and at night, under warm clothes and in bed. In this case, the skin is not changed at first, scratching appears later. Dry eyes and mouth, pain in the right hypochondrium, and joint pain may cause discomfort. Later, jaundice, darkening of the skin, yellowish cholesterol deposits around the eyes, and signs of osteoporosis appear.
- Severe fatigue
- Itchy skin that gets worse at night
- Dry eyes and mouth
- Discomfort in the right hypochondrium
- Joint pain
- Jaundice and darkening of the skin in later stages
- Bone fragility and fractures
Diagnostics
A persistent increase in alkaline phosphatase and GGT with moderately altered ALT and AST allows one to suspect the disease. The key test is antimitochondrial antibodies, which are found in the vast majority of patients; in their absence, specific antinuclear antibodies are examined. Ultrasound and other imaging methods are needed to exclude mechanical obstruction to the outflow of bile, stones and tumor. A liver biopsy is not always required, mainly if the picture is unclear or a combination with autoimmune hepatitis is suspected. The degree of fibrosis is assessed by elastography.
- Alkaline phosphatase, GGT, bilirubin, ALT and AST
- Antimitochondrial antibodies
- Immunoglobulin M and antinuclear antibodies
- Ultrasound of the liver and biliary tract
- Liver elastography
- Liver biopsy according to indications
- Densitometry to assess bone density
Treatment
The basic drug is ursodeoxycholic acid, which is taken long-term, usually for life. It improves bile flow, reduces biochemical parameters and has been proven to slow progression, increasing life expectancy without transplantation. The response to therapy is assessed after 6–12 months by tests; if the effect is insufficient, the doctor adds second-line drugs. Symptoms are treated separately: itching is reduced with bile acid binders and other drugs as prescribed by the doctor. Prevention of osteoporosis, replenishment of vitamin D and calcium, and avoidance of alcohol are mandatory. For decompensated cirrhosis, liver transplantation is discussed.
- Ursodeoxycholic acid long-term
- Evaluation of response to therapy after 6–12 months
- Second-line drugs with insufficient effect
- Treatment of skin itching as prescribed by a doctor
- Vitamin D, calcium, prevention of osteoporosis
- Complete abstinence from alcohol
- Vaccination against hepatitis A and B
- Liver transplantation for decompensation
Observation and lifestyle
The disease requires regular monitoring: biochemical parameters are checked several times a year, assessment of fibrosis and ultrasound of the liver - according to a schedule determined by the doctor. In case of cirrhosis, monitoring for varicose veins of the esophagus and screening for liver tumors are added. In everyday life, avoiding alcohol, being careful with medications and herbal supplements that can damage the liver, eating a nutritious diet with adequate protein, and moderate physical activity are important. Fatigue is facilitated by a daily routine with distribution of loads and short breaks.
- Monitoring blood biochemistry several times a year
- Periodic elastography and ultrasound of the liver
- Screening for varicose veins and tumors in cirrhosis
- Avoidance of alcohol and hepatotoxic additives
- Adequate amounts of protein and vitamin D
- Moderate physical activity
- Planning pregnancy only with a doctor