Definition of primary biliary cirrhosis
Definition of primary biliary cirrhosis
Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease of small bile ducts located within the liver (called intrahepatic bile ducts). CBP is of unknown cause and occurs in women after age 40.
Doctors use the term "cirrhosis" even if this does not occur immediately. But like all liver cirrhosis, CBP leads to a gradual destruction of liver cells (called hepatocytes) and eventually it is replaced by fibrosis that does not allow it to perform its functions. The diagnosis is based on liver biopsy or liver biopsy (LB).
Risks and consequences of biliary cirrhosis
More than 10,000 cases are reported, which represents a prevalence of 20 per 100 000 inhabitants. CBP preferentially affects women over 40-45 years, with a prevalence of up to 86 home for 100,000.
Genetic factors and environmental factors (smoking, infections, chemicals ...) explain the disease.
Causes and origins of cirrhosis
PBC is characterized primarily by an inflammatory bile duct located within the liver. This portal inflammation and cell death (called necrosis) result in stasis syndrome (or cholestasis) that autoentretient phenomenon. Autoimmune cause is suspected, because of autoantibodies that destroy cells are almost always highlighted.
Cirrhosis appears only later, with lesions of liver tissue that fibrosent. This stage of cirrhosis is irreversible.
Various forms of cirrhosis according to the speed of evolution:
- The classical form leads to biliary cirrhosis in 10 to 20 years;
- The form of cirrhosis called "overlap syndrome" is more severe, with a change in less than 10 years;
- Form severe cirrhosis changing in less than 5 years.
Primary biliary cirrhosis Prevention
How does this manifest itself on?
CBP can long remain asymptomatic and the diagnosis is then suspected that during laboratory testing. The present some symptoms are nonspecific, such as pruritus (itching) or fatigue. Some patients are diagnosed when the balance of another disease (autoimmune).
In more severe or advanced symptoms associated with cirrhosis are present, including jaundice. Jaundice is a coloring of the skin and mucous membranes.
In some cases, the signs are those of hepatitis with jaundice, pain, fever and severe abnormalities.
With what should we be confused?
It should not be confused CBP and alcoholic cirrhosis. Alcoholic cirrhosis is actually the most common, but other causes of cirrhosis are as hepatitis.
Is there a prevention possible?
There is no prevention of CBP. Environmental factors can only be limited to: alcoholic abstinence, prevention of hepatitis C (intravenous drug use) and B (sexual transmission).
In cirrhosis proved must prevent bacterial infections (oral care, skin hygiene ...). Any infection should be treated quickly because it can lead to decompensation of cirrhosis.
Vaccinations are recommended, such as those against hepatitis A, hepatitis B, influenza and pneumococcus.
Primary biliary cirrhosis Preparing consultation
When to consult?
As the disease is diagnosed early and treated, the better the chances of stabilization (but not healing because CBP is an irreversible process).
The elimination of risk cofactors, including alcohol, is essential. Regular monitoring of liver function is imperative to detect and monitor complications of the disease.
What is the doctor?
With cirrhosis, the doctor finds the presence of a large liver palpating the abdomen, it also checks for other signs pointing to cirrhosis (angiomas or wine stains, redness, abnormal vessels on the stomach ...). Signs of complications are also searched: earthquakes encephalopathy, signs of gastrointestinal bleeding ...
Depending on the status and form of the disease, different abnormalities are found: signs of cholestasis (bile retention) and signs of liver destruction (or cytolysis) are key. Other tests can both determine the stage of the disease and its impact (coagulation, platelets ...), but also to eliminate other causes (hepatitis serology, criteria for alcoholism ...).
The diagnosis of PBC is strongly suspected the presence of antibodies called mitochondrial (AMA M2 type). It will be confirmed by liver biopsy that reveals inflammatory lesions (or lymphocytic destructive cholangitis).
Treatment is based on ursodeoxycholic acid (UDCA), which slows the progression of fibrosis, and corticosteroids in some cases (signs of autoimmune hepatitis).
At an advanced stage of the disease, the only solution is liver transplantation.
How to prepare my next visit?
Psychological support in networks of care or patient associations can help patients.
Any infection in cirrhotic patients requires rapid consultation.
Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease of small bile ducts located within the liver (called intrahepatic bile ducts). CBP is of unknown cause and occurs in women after age 40.
Doctors use the term "cirrhosis" even if this does not occur immediately. But like all liver cirrhosis, CBP leads to a gradual destruction of liver cells (called hepatocytes) and eventually it is replaced by fibrosis that does not allow it to perform its functions. The diagnosis is based on liver biopsy or liver biopsy (LB).
Risks and consequences of biliary cirrhosis
More than 10,000 cases are reported, which represents a prevalence of 20 per 100 000 inhabitants. CBP preferentially affects women over 40-45 years, with a prevalence of up to 86 home for 100,000.
Genetic factors and environmental factors (smoking, infections, chemicals ...) explain the disease.
Causes and origins of cirrhosis
PBC is characterized primarily by an inflammatory bile duct located within the liver. This portal inflammation and cell death (called necrosis) result in stasis syndrome (or cholestasis) that autoentretient phenomenon. Autoimmune cause is suspected, because of autoantibodies that destroy cells are almost always highlighted.
Cirrhosis appears only later, with lesions of liver tissue that fibrosent. This stage of cirrhosis is irreversible.
Various forms of cirrhosis according to the speed of evolution:
- The classical form leads to biliary cirrhosis in 10 to 20 years;
- The form of cirrhosis called "overlap syndrome" is more severe, with a change in less than 10 years;
- Form severe cirrhosis changing in less than 5 years.
Primary biliary cirrhosis Prevention
How does this manifest itself on?
CBP can long remain asymptomatic and the diagnosis is then suspected that during laboratory testing. The present some symptoms are nonspecific, such as pruritus (itching) or fatigue. Some patients are diagnosed when the balance of another disease (autoimmune).
In more severe or advanced symptoms associated with cirrhosis are present, including jaundice. Jaundice is a coloring of the skin and mucous membranes.
In some cases, the signs are those of hepatitis with jaundice, pain, fever and severe abnormalities.
With what should we be confused?
It should not be confused CBP and alcoholic cirrhosis. Alcoholic cirrhosis is actually the most common, but other causes of cirrhosis are as hepatitis.
Is there a prevention possible?
There is no prevention of CBP. Environmental factors can only be limited to: alcoholic abstinence, prevention of hepatitis C (intravenous drug use) and B (sexual transmission).
In cirrhosis proved must prevent bacterial infections (oral care, skin hygiene ...). Any infection should be treated quickly because it can lead to decompensation of cirrhosis.
Vaccinations are recommended, such as those against hepatitis A, hepatitis B, influenza and pneumococcus.
Primary biliary cirrhosis Preparing consultation
When to consult?
As the disease is diagnosed early and treated, the better the chances of stabilization (but not healing because CBP is an irreversible process).
The elimination of risk cofactors, including alcohol, is essential. Regular monitoring of liver function is imperative to detect and monitor complications of the disease.
What is the doctor?
With cirrhosis, the doctor finds the presence of a large liver palpating the abdomen, it also checks for other signs pointing to cirrhosis (angiomas or wine stains, redness, abnormal vessels on the stomach ...). Signs of complications are also searched: earthquakes encephalopathy, signs of gastrointestinal bleeding ...
Depending on the status and form of the disease, different abnormalities are found: signs of cholestasis (bile retention) and signs of liver destruction (or cytolysis) are key. Other tests can both determine the stage of the disease and its impact (coagulation, platelets ...), but also to eliminate other causes (hepatitis serology, criteria for alcoholism ...).
The diagnosis of PBC is strongly suspected the presence of antibodies called mitochondrial (AMA M2 type). It will be confirmed by liver biopsy that reveals inflammatory lesions (or lymphocytic destructive cholangitis).
Treatment is based on ursodeoxycholic acid (UDCA), which slows the progression of fibrosis, and corticosteroids in some cases (signs of autoimmune hepatitis).
At an advanced stage of the disease, the only solution is liver transplantation.
How to prepare my next visit?
Psychological support in networks of care or patient associations can help patients.
Any infection in cirrhotic patients requires rapid consultation.
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