就诊准备指南：实用手册

Overview

Primary biliary cholangitis (PBC) is a chronic autoimmune disease that slowly damages the small bile ducts inside your liver. When these ducts are damaged, bile (a fluid that helps digest food) builds up in the liver, causing inflammation and gradual scarring over time. PBC was previously called primary biliary cirrhosis, but the name was updated because not everyone with the condition develops cirrhosis (advanced liver scarring). It most often affects people assigned female at birth, and is usually diagnosed between the ages of 30 and 60. With early and consistent treatment, most people with PBC can slow disease progression and live full, active lives.

Signs & Symptoms

Many people with early PBC have no noticeable symptoms, and the condition is often first detected via routine blood tests showing abnormal liver enzymes. When symptoms do appear, they vary widely from person to person, and may include: persistent fatigue that is not relieved by rest; itchy skin (pruritus), which may be mild or severe; dry eyes and mouth (sicca symptoms); yellowing of the skin and eyes (jaundice) in more advanced cases; pain in the upper right abdomen; dark urine; and pale, clay-colored stools.

Causes & Risk Factors

The exact cause of PBC is not fully understood, but it is classified as an autoimmune condition: this means your immune system mistakenly attacks the healthy cells and tissues of the small bile ducts in your liver, leading to damage over time. Risk factors for developing PBC include: being assigned female at birth (around 90% of cases occur in women); having a family history of PBC or other autoimmune conditions (such as rheumatoid arthritis, Sjögren’s syndrome, or autoimmune thyroid disease); smoking; and possible exposure to certain environmental triggers like infections or industrial chemicals. PBC is not contagious, and is not caused by poor diet or lifestyle choices alone.

Diagnosis

If your doctor suspects PBC based on symptoms or abnormal liver test results, they will order a series of tests to confirm the diagnosis and rule out other liver conditions. Most people are diagnosed via a combination of blood tests, imaging, and sometimes a liver biopsy.

Treatment & Management

There is no cure for PBC, but treatments can slow or stop disease progression, manage symptoms, and prevent serious complications. The first-line treatment for most people is ursodeoxycholic acid (UDCA), a safe, oral bile acid that helps move bile out of the liver and protect liver cells from further damage. For people who do not respond well to UDCA, additional medications like obeticholic acid may be prescribed. Symptom-specific treatments are also available: for example, cholestyramine or rifampicin for severe itching, and lubricating eye drops for dry eyes. Regular monitoring of liver function and screening for complications like cirrhosis, portal hypertension, and liver cancer is a critical part of long-term care.

Living With It

PBC is a chronic, lifelong condition, but with consistent care and management, most people with early-treated PBC can expect to live full, active lives for many decades. Key parts of long-term management include regular follow-up appointments with a hepatologist (a liver specialist), protecting your liver from additional harm, and addressing the emotional impact of living with a chronic illness.

• Avoid alcohol completely, as it can worsen liver inflammation and damage
• Limit use of over-the-counter pain relievers like acetaminophen, which can cause liver damage in high doses
• Eat a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall liver health
• Stay physically active with low-impact exercise like walking or yoga as tolerated, to reduce fatigue and maintain a healthy weight
• Get vaccinated against hepatitis A and B, influenza, and other recommended illnesses to protect your liver from additional stress