PBC is a chronic autoimmune liver disease in which the immune system attacks the bile ducts, causing their damage. To understand the primary biliary cholangitis, we need to know what is the bile ducts and bile.
Bile ducts are small tubes that drain the bile juice from the liver to the small intestine. Bile is a yellowish-green fluid that the liver makes, and the bile ducts take to the gall bladder and the small intestine. Bile helps you digest fat and cholesterol and absorb the fat-soluble vitamins (A, D, E, and K). It also helps your body get rid of toxins and aged red blood cells.
In PBC, the immune system causes inflammation and progressive damage to the bile duct and blocks bile drainage. This block leads to the accumulation of bile and other substances in the liver. Bile and these substances are very irritant and harmful to the liver cells, which causes the disease. In PBC, the bile acids damage the liver and can’t function in the small intestine.
PBC is a rare condition; it affects one person per 3000 to 4000 people. It is an autoimmune disease, and -as most of them- it affects women more than men. It affects both sexes at the ratio of 9 women to 1 man. It affects women between 40 to 60 years old. PBC has no known causes; we only know some predisposing factors and triggers.
The presentation of PBC ranges from asymptomatic to liver cirrhosis and its complications. The common manifestations are nonspecific, such as fatigue and itching. As with most autoimmune diseases, when you have PBC, you may have another autoimmune disease.
Diagnosis of PBC is difficult in the early stages because there may be no or nonspecific symptoms. Thus, it may occur during routine tests or investigating another problem. The diagnosis begins with suspicion of a liver problem from the history and examination, then the laboratory tests confirm it.
There is no curative treatment for PBC other than liver transplantation, which is the last option. But, your doctor will put a management plan to slows the disease progression, treat the symptoms, and prevent more complications.
Now, let’s see this disease in more detail. We will discuss the following:
- What causes this disease, and who is at risk of having it?
- What are its symptoms, signs, and advanced complications?
- How will my doctor diagnose if I have this disease and confirm this diagnosis?
- How can my doctor treat me, and what are the treatment options that I have?
Causes and risk factors of PBC
From its name (primary biliary cholangitis), it is a primary disease that the scientists didn’t know what exactly causes it. But, they think that genetic and environmental factors may trigger it. Also, some risk factors may put you at a higher risk, and they include:
- Female sex (9 females: 1 male)
- Age: PBC is more common at the age of 40 to 60 years.
- Positive family history: Having a 1st-degree relative who has or had PBC puts you at significant risk to develop it.
- Race: It occurs in all races, but it is more common in white north Europeans.
Scientists think that some environmental factors trigger the disease when they combine with a genetic predisposition. These triggers include infections (such as urinary tract infections), smoking, and toxic chemicals.