Primary biliary cirrhosis is classified as an autoimmune disease, which means that the body attacks its own tissues. A characteristic symptom of the disease is the feeling of chronic fatigue and nagging itching of the skin. Find out about the treatment of primary biliary cirrhosis and check the prognosis.

Primary biliary cirrhosisis an increasingly recognized disease. In the 1970s, its incidence was estimated at approx. 40 cases per million, currently at approx. 250-350 cases per million. More than 90% of patients are women, whose first symptoms usually appear in the age of 30-60. Primary biliary cirrhosis does not occur in children.

Primary biliary cirrhosis - causes

The underlying cause of the disease is the many years of damage to cholangiocytes, i.e. bile duct epithelial cells. This process leads to an impairment of the liver's bile secretion. The obstructed outflow of bile from the liver consequently leads to serious damage to the organ known as cholestasis.

The name primary biliary cirrhosis does not reflect the essence of the disease, but such a term was adopted in the 1950s.

Modern diagnostic possibilities allow to detect the disease at an early stage of the disease development. This often happens long before the changes appear on histological examination, that is, when examining tissues under a microscope. In other words, changes can be detected before advanced liver damage, i.e., before cirrhosis develops.

Primary biliary cirrhosis - symptoms

The main clinical symptoms include the feeling of chronic fatigue and nagging itching of the skin.

A feature of chronic fatigue in primary biliary cirrhosis of the liver is that it does not increase significantly after increased physical exertion, but also does not subside after rest. The feeling of fatigue constantly accompanies the patient.

Skin itching can vary in severity, from moderate itching in the hands and feet to severe itching throughout the body.

In some patients, primary biliary cirrhosis may be completely asymptomatic or only causenon-specific pain in the right hypochondrium.

As lipid metabolism is disturbed in the course of the disease, some patients may develop skin changes at the base of the nose. This is called yellows or yellow tufts clearly visible under the skin. Jaundice is a benign histiocytic tumor. They are flat, yellow protrusions. They are filled with cholesterol and other fat bodies. They don't tend to be angry. They are usually surgically removed for cosmetic reasons.

Patients with primary biliary cirrhosis quite often also suffer from other autoimmune diseases and have clinical symptoms of these diseases. We include:

  • Sjögren's team
  • autoimmune diseases of the thyroid gland
  • rheumatoid arthritis
  • scleroderma

In a small group of patients, primary biliary cirrhosis is diagnosed in the stage of advanced liver damage. Symptoms may then occur:

  • of destruction
  • jaundice
  • ascites
  • swelling of the lower limbs
  • bruising

Primary biliary cirrhosis - diagnosis

Chronic fatigue is not a typical symptom that can be easily explained by overwork. But the itching of the skin is hard to miss. When such symptoms occur, contact your primary care physician.

Unfortunately, it happens that patients with itchy skin are referred to a dermatologist, which significantly prolongs the correct diagnosis. It may also deteriorate the general he alth of the patient.

Therefore, the physician should order basic biochemical tests of the liver (alkaline phosphatase and γ-glutamyltranspeptidase - GGT activity tests).

The diagnosis of primary biliary cirrhosis is considered reliable if the following 3 criteria are met (likely if two are present):

  1. increased activity of alkaline phosphatase, an enzyme indicative of bile outflow disorders
  2. presence of anti-mitochondrial antibodies in the serum (highly specific for primary biliary cirrhosis)
  3. a typical image of a sample taken during a liver biopsy (i.e. a liver puncture), showing, inter alia, features of biliary damage

Primary biliary cirrhosis - treatment

Treatment of primary biliary cirrhosis should be handled by a hepatologist, a doctor who specializes in treating liver disease. The primary goal of treatment is to slow the progression of the disease and to eliminate it or significantly weaken itclinical symptoms.

Patients are given ursodeoxycholic acid, which improves the drainage of bile from the liver, thus reducing its toxic effects on the liver. This effectively delays disease progression in many patients.

In symptomatic treatment, preparations reducing the severity of itching of the skin are used. The drug of choice is cholestyramine.

Patients suffering additionally from Sjögren's syndrome develop dry mouth and dry conjunctiva. In such cases, it is recommended to drink small amounts of water frequently and use the so-called artificial tears that moisturize the surface of the eyes.

In people who have had significant liver damage, including liver failure, organ transplantation is the only effective treatment.

Primary biliary cirrhosis treatment is chronic.

Follow your doctor's recommendations, take care of a proper diet, avoid the consumption of alcohol and drugs that are toxic to the liver.

Patients with a transplanted liver must take medications to prevent transplant rejection.

Primary biliary cirrhosis - prognosis

Unfortunately, they're not good. It is widely believed that a complete recovery of primary biliary cirrhosis is not possible. But early diagnosis and appropriate treatment are very effective in slowing disease progression in most patients.

It can be said that by following the doctor's recommendations, patients with primary biliary cirrhosis survive as much as he althy people.

The prognosis is very good in patients who require liver transplantation. Over 80% of patients survive for at least 5 years after surgery. Although a transplanted liver can relapse of primary biliary cirrhosis, the condition does not appear to have serious consequences in most patients.

About the authorAnna Jarosz A journalist who has been involved in popularizing he alth education for over 40 years. Winner of many competitions for journalists dealing with medicine and he alth. She received, among others The "Golden OTIS" Trust Award in the "Media and He alth" category, St. Kamil awarded on the occasion of the World Day of the Sick, twice the "Crystal Pen" in the national competition for journalists promoting he alth, and many awards and distinctions in competitions for the "Medical Journalist of the Year" organized by the Polish Association of Journalists for He alth.

Read more articles by this author

Category:

Gastrology