Why are women more likely to suffer from primary cholangitis? The science behind it is revealed!

Primary cholangitis (PBC), once known as primary biliary cirrhosis, is an autoimmune liver disease. This disease causes the gradual destruction of the small bile ducts in the liver, causing bile and other toxins to accumulate in the liver, which may eventually lead to cirrhosis. According to research, the incidence of PBC in women is much higher than that in men, with a gender ratio as high as 9:1. Why is this? Let’s discuss it together.

Cause analysis

The pathological basis of primary cholangitis is related to the immune system. Most patients have anti-mitochondrial antibodies (AMA). These antibodies attack specific enzyme complexes in liver cells, causing damage to small bile ducts.

It's unclear why women are more likely to develop PBC, but some research suggests that sex hormones such as estrogen may play an important role in the immune system's response. Some scholars have pointed out that in autoimmune diseases, estrogen seems to increase the risk of autoimmune reactions.

Symptoms and diagnosis

Patients with PBC often feel tired (about 80%), which is a non-specific symptom that has a profound impact on the quality of life. Other common symptoms include itchy skin (20%-70% of patients), which is often not associated with progression of liver disease. Many patients may only have liver function abnormalities found in routine blood tests in the early stages without obvious symptoms.

The diagnosis of primary cholangitis mainly relies on specific serum markers, such as the detection of anti-mitochondrial antibodies. 90%-95% of PBC patients will develop this antibody.

Genetic factors

Genetics also play an important role in the development of PBC. Multiple studies have shown a tendency for the disease to cluster among family members, and comorbidity rates among twins also suggest underlying genetic susceptibility. A 2012 genomics study revealed 26 genetic loci associated with PBC that influence the regulation of cytokines.

Environmental factors and bacterial infection

In addition to genetic factors, environmental factors may also influence the development of PBC. Some studies suggest that environmental bacteria, such as Novosphingobium aromaticivorans, may be involved in autoimmune responses. Proteins from these bacteria may cross-react with mitochondrial proteins in liver cells, further exacerbating the immune system's attack.

Treatment Plan

Currently, the treatment for PBC is mainly based on drug control. The first-line therapy is ursodeoxycholic acid (UDCA). For patients who have poor response to UDCA, obeticholic acid (OCA) can be considered. These two drugs can effectively improve liver function indicators and slow down the progression of the disease.

The introduction of UDCA has completely changed the treatment model of PBC and significantly improved the survival rate of patients.

Looking to the future

Although current treatments have improved for many patients, targeted therapies are still lacking for the 80% of patients who report symptoms of fatigue. Researchers continue to explore new drugs and treatments, hoping to provide more treatment options to improve patients' quality of life in the future.

As our understanding of PBC deepens and research continues to advance, can we find more effective strategies to solve this disease, not only targeting symptoms, but also fundamentally improving women's health and well-being?

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