What is primary cirrhosis
Primary cirrhosis (PBC) is a chronic autoimmune liver disease that mainly affects the small bile ducts in the liver, leading to cholestasis, liver fibrosis, and eventually cirrhosis. The disease mostly occurs in middle-aged women. The cause is not yet completely clear and may be related to genetic, environmental and immune factors. The following is a detailed analysis of primary cirrhosis.
1. Causes and pathogenesis of primary liver cirrhosis
The specific cause of primary cirrhosis is unknown, but research suggests that the following factors may be related to its onset:
| possible causes | Description |
|---|---|
| genetic factors | Patients with family history have a higher incidence rate, and some genetic variations (such as HLA-DRB1*08) are associated with PBC. |
| immune abnormalities | Autoantibodies (such as anti-mitochondrial antibody AMA) attack bile duct epithelial cells and trigger an inflammatory response. |
| environmental factors | Smoking, infections (such as urinary tract infections), and exposure to chemicals may trigger illness. |
2. Clinical manifestations of primary liver cirrhosis
The early symptoms of PBC are often not obvious. As the disease progresses, the following symptoms may appear:
| Symptom classification | Specific performance |
|---|---|
| early symptoms | Fatigue, skin itching (bile acid deposition), dry mouth and eyes (combined with Sjogren's syndrome). |
| Late symptoms | Complications related to jaundice, ascites, hepatosplenomegaly, osteoporosis, and portal hypertension. |
3. Diagnosis and examination
The diagnosis of PBC requires a combination of clinical manifestations, laboratory tests and imaging results:
| Check items | meaning |
|---|---|
| blood test | Anti-mitochondrial antibody (AMA-M2) is positive (more than 95% of patients), and alkaline phosphatase (ALP) is elevated. |
| Imaging examination | Ultrasound or MRI excludes bile duct obstruction, and liver elastography assesses the degree of fibrosis. |
| liver biopsy | The gold standard for diagnosis shows bile duct destruction and inflammatory cell infiltration. |
4. Treatment plan
There is currently no cure for PBC, but early intervention can delay the progression of the disease:
| Treatment | Specific measures |
|---|---|
| drug treatment | Ursodeoxycholic acid (UDCA) is used as first-line medication, and obeticholic acid (OCA) is used for patients in whom UDCA is ineffective. |
| Symptomatic treatment | Antipruritic drugs (such as cholestyramine) and vitamin D supplementation prevent osteoporosis. |
| liver transplant | The only effective method for end-stage patients, the 5-year survival rate can reach 70%-80%. |
5. Prevention and prognosis
The prognosis of PBC is closely related to the timing of diagnosis:
| prognostic factors | influence |
|---|---|
| early diagnosis | The 10-year survival rate can reach over 80% (for those who have not progressed to cirrhosis). |
| late diagnosis | After liver cirrhosis occurs, the median survival period is about 5-8 years. |
In terms of prevention, avoiding smoking and controlling infections may reduce the risk, and regular physical examinations (especially those with a family history) can help with early detection.
6. Hot topics in the past 10 days
Recent discussions about primary cirrhosis have focused on:
| hot topics | Summary of contents |
|---|---|
| New drug research and development | Phase III clinical trial data of PPAR agonists (such as Elafibranor) were released and are expected to become new treatments. |
| patient quality of life | Fatigue management guidelines updated, recommending cognitive behavioral therapy combined with exercise intervention. |
| Improve disease awareness | The International PBC Awareness Week emphasizes the awareness of early screening that “itching is not a trivial matter”. |
In summary, primary cirrhosis is a chronic disease that requires long-term management. Early diagnosis and standardized treatment can significantly improve the prognosis. The public should pay attention to liver health signals, conduct regular screening of high-risk groups, and implement scientific prevention and treatment.
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