The liver defends itself: What's the mystery of primary cholangitis?

Primary Biliary Cholangitis (PBC) is an autoimmune liver disease characterized by the chronic, progressive destruction of the small bile ducts in the liver. As the condition progresses, bile and other toxins build up in the liver, causing cholestasis. This process not only leads to chronic damage to liver tissue, but may eventually cause fibrosis and cirrhosis. According to statistics, the incidence of PBC is approximately one in every 3,000 to 4,000 people, and it is more common in females, with a gender ratio of up to 9:1.

Early-stage PBC may only show abnormalities in blood tests without showing any obvious symptoms.

The cause of PBC is still unclear, but researchers speculate that it may involve the expression of sex hormones such as estrogen, which affects the response of the immune system to some extent. The clinical manifestations of PBC are often accompanied by fatigue, itchy skin, and jaundice in more advanced stages, which significantly affect the patient's quality of life. Generally speaking, the incidence of PBC in women is higher than that in men, but the specific reasons still need further study.

Symptoms and Diagnosis

Approximately 80% of PBC patients experience fatigue, a nonspecific symptom that can lead to a significant decrease in quality of life. Dry skin and eyes are also common symptoms, with pruritus of varying intensity experienced by 20% to 70% of patients. The itching is often worse at night, and symptoms are not necessarily related to the progression of liver disease.

Studies have shown that itching in PBC patients can occur even when liver function tests are normal.

Most PBC patients today are diagnosed when blood tests are done in the absence of symptoms, primarily because of abnormal liver enzyme tests—such as gamma-glutamyl transpeptidase (GGT) or alkaline phosphatase (ALP). Increase. The key to diagnosing PBC is to detect anti-mitochondrial antibodies (AMA) in the blood. This antibody plays an important role in mitochondria and is present in about 90% to 95% of PBC patients.

Treatment and Management

The treatment of PBC mainly focuses on controlling disease progression and relieving symptoms. The current first-line drug is udarachidonic acid (UDCA), which can improve liver enzyme levels and slow the progression of the disease. For patients who do not respond to UDCA, further treatment with other drugs such as obeticholic acid (OCA) may be an option.

The use of UDCA has significantly changed the treatment paradigm for PBC patients over the past few decades.

In the study, patients who did not improve during UDCA treatment had a significantly increased risk of future hepatocellular carcinoma (HCC). Therefore, it is very important for patients with PBC to regularly track liver function and use appropriate treatment methods.

Prognosis and Lifestyle Adjustment

With the deepening of understanding of PBC and the improvement of treatment methods, the survival rate of patients has been significantly improved. If the patient's liver enzyme levels return to normal range after using UDCA, the prognosis is relatively good, and the survival rate can even reach the level of the general population. In terms of lifestyle, PBC patients should try to avoid alcohol intake and undergo regular physical examinations, especially for screening and management of complications such as osteoporosis.

Although PBC is a relatively rare disease, its impact on patients' lives cannot be underestimated. Early diagnosis, timely treatment and continuous monitoring of such diseases are the key to improving prognosis. The active participation of patients and close cooperation with the medical team will gradually reveal the mystery of this disease. How can we better understand and treat PBC in the future to improve patients' quality of life?

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