Primary biliary cirrhosis

Primary biliary cirrhosis (PBC, chronic non-suppurative destructive cholangitis ) is a relatively rare autoimmune disease of the liver that affects women in about 90 % of cases. This begins (primary ) to the small bile ducts ( biliary ) that are destroyed by inflammation. In the long course of the inflammation can spread to the whole liver tissue and eventually lead to scarring through to cirrhosis. The name " cirrhosis " is misleading, since not everyone PBC patient at the time of diagnosis already has cirrhosis. The term dates back to times when the PBC could be detected only in the final stage. Meanwhile, can the disease ( eg, by the detection of anti-mitochondrial antibodies, AMA in the blood) already demonstrated, when the liver is still relatively intact. Nevertheless, the stages are called before reaching the PBC cirrhosis in clinical parlance.

Symptoms

Especially common symptoms of PBC are fatigue and exhaustion (70-90 % of patients ) and pruritus ( 20-70 %). Rheumatism -like side effects include joint pain, thyroid disease ( Hashimoto 's thyroiditis ) and dry mucous membranes ( sicca syndrome ). Small fatty deposits in the inner corners of eyes ( xanthelasma ) are observed in approximately 20 % of patients. Steatorrhea and vitamin deficiency (especially vitamin A, D, E and K ) may also be associated with PBC. At 20 % of women with PBC occurring (recurrent ) urinary tract infections have been found again and again. In the late stage of cirrhosis can be set typical for cirrhosis complications (see cirrhosis). These include water belly ( ascites), varicose veins in the esophagus ( esophageal varices ) or in the stomach ( fundus varices ), brain function disorders (hepatic encephalopathy ) and liver cancer. Whether the PBC also increases the risk of bone loss (osteoporosis), but is provided by new research results into question.

Diagnosis

For most patients with PBC, the so-called anti-mitochondrial antibodies (AMA ) are elevated in the blood. This finding, the diagnosis can often prove so. ( A rare exception without AMA increase the so-called " negative AMA PBC", which is also referred to as Autoimmuncholangitis. )

General laboratory values ​​, which may indicate bile duct inflammation or cholestasis, also often lie above the normal range: these include alkaline phosphatase ( AP) and gamma -glutamyl transpeptidase (GGT or gamma - GT). The protein compound IgM (antibodies) can be also increased by PBC.

In ultrasound ( sonography), the liver can unobtrusively or look similar to a fatty liver at an early stage. In later stages, the liver may be enlarged, in end-stage cirrhosis, the surface is often bumpy or wavy, the liver can shrink again here.

A liver biopsy (liver biopsy) may help in the diagnosis, secure the diagnosis also has a tissue examination.

It is important that PBC clear of other autoimmune diseases such as Autoimmune hepatitis or primary sclerosing cholangitis delineate. In up to 10% of cases mixed methods may, for example, of PBC and autoimmune hepatitis occur (so-called overlap syndrome ).

Causes

The causes and triggers of PBC are now known. Studies suggest that it is an autoimmune disease. This means that your own immune system due to a defect can no longer distinguish between " foreign " and " self " and attacks the mitochondria in body cells. The corresponding autoantibodies against the E2 subunit of the pyruvate dehydrogenase complex, the enzyme directed dihydrolipoyl transacetylase.

Strong dissent it, what other factors play a PBC can bring to the outbreak there. It discusses, inter alia, hormonal and genetic influences, medications, infections with viruses, fungi or bacteria, and environmental influences. One study pointed out that E2 derivatives in which lipoic acid has been replaced by octyne - 7-carboxylic acid, a methyl esters industrially as a fragrance and flavoring ( Violet) compound used, the modified E2 had a much stronger antigenicity. Allergies to the methyl esters are known. Cosmetic products with this material must be identified across the EU ( " METHYL 2- Octynoate ").

The influence of pregnancy on PBC and vice versa of PBC on pregnancy has not been established. Alcohol is known to be no trigger of PBC. However, it can adversely affect the course as with all liver disease and should be strictly avoided.

Today's therapy

If left untreated, a PBC lead within about twelve years to death, but of course from patient to patient is very different and in the early stages of the disease, life expectancy does not seem to be much more limited. The standard treatment of primary biliary cirrhosis is today ursodeoxycholic acid ( UDCA ), which can be given as a tablet. The therapy is usually well tolerated, is after diagnosis, and lasts a lifetime. The goal of therapy is to slow down the progression of the disease and to improve laboratory values ​​.

Transplantation

The disease progresses despite therapy to decompensated cirrhosis or advanced symptoms such as Unbearable itching despite treatment for patients, a liver transplant is often necessary. In 75% of transplant patients, the PBC is thus cured at 25 % can again develop PBC -like damage in the new liver. After a successful liver transplantation, the long-term prognosis of patients with PBC is good.

Future prospects

A breakthrough in the search for causes of PBC is expected to continue in the coming years, hardly. Many studies seem in itself plausible, but contradictory in the results. Also reports in the medical press, the cause is found (eg environmental influences or retroviruses ), should be taken with great caution.

In order to improve the treatment of PBC on, is currently being investigated in studies whether the combination of ursodeoxycholic acid ( UDCA ) with corticosteroids more effective than UDCA alone. Final results are pending, approved the combination therapy in Germany is not currently (as of May 2008).

Gallery

Photomicrograph of primary biliary cirrhosis ( H & E, low magnification).