2021 Standard for the diagnosis and treatment of primary biliary cholangitis, take a look at the latest guidelines!

Primary biliary cholangitis ( PBC ) is a chronic progressive autoimmune cholestatic disease with small hepatic bile ducts as the main target organ.
Fibrosis and liver cirrhosis
.

Primary biliary cholangitis ( PBC ) is a chronic progressive autoimmune cholestatic disease with small hepatic bile ducts as the main target organ.
Fibrosis and liver cirrhosis
.

immunity

PBC is not uncommon in China , but the level of standardized diagnosis and treatment still needs to be improved.
It is of great significance to standardize the clinical diagnosis and treatment of PBC in China to formulate the diagnosis and treatment of primary biliary cholangitis based on clinical practice in China .
This article organizes the core points of the " 2021 Guidelines for the Diagnosis and Treatment of Primary Biliary Cholangitis", including the clinical characteristics, treatment options and prognosis of PBC , to provide guidance and decision-making basis for clinicians .

PBC is not uncommon in China , but the level of standardized diagnosis and treatment still needs to be improved.

On the basis of clinical practice in China, formulating the diagnosis and treatment standard of primary biliary cholangitis is of great significance to standardize the clinical diagnosis and treatment of PBC in China.
.

1.
Clinical features of PBC 1.

With the improvement of the understanding of PBC and the level of clinical diagnosis, the prevalence of PBC in China is on the rise

In the early stage, the vast majority of PBC patients had no obvious clinical symptoms, and some had symptoms such as itching and fatigue

Preclinical stage: This stage may only be positive for autoantibodies such as high titer anti-mitochondrial antibodies ( AMA ) in serum, and there is no abnormal serum biochemical index.

Asymptomatic stage: This stage is characterized by abnormal biochemical indicators and no obvious clinical symptoms.

Symptomatic stage: At this stage, clinical manifestations the time from symptom onset to progression to liver cirrhosis is about 5 to 8 years

Decompensation stage: In this stage, decompensation of liver cirrhosis , and the survival period may be less than 3 years

Common complications of PBC include cholestasis-related and extrahepatic organ involvement, associated diseases involving autoimmune diseases and liver malignancies.
According to studies, the risk of hepatocellular sarcoma in PBC patients is significantly higher than that in healthy people, especially those with liver cirrhosis.

2.
Diagnosis of PBC 2.

In the process of diagnosis, PBC needs to be differentiated from drug-induced cholestasis, alcoholic liver cirrhosis, obstructive cholestasis, sarcoidosis, AIH , PSC and other diseases , and at the same time, it is necessary to screen for the presence of other systemic connective tissue screening diseases or other diseases.

If two of the following three conditions are met , a diagnosis of PBC can be made :

1.
Elevated alkaline phosphatase ( ALP ) and other serum biochemical evidence reflecting cholestasis
.

1.
Elevated alkaline phosphatase ( ALP ) and other serum biochemical evidence reflecting cholestasis
.

2.
High titer of anti-mitochondrial antibody ( AMA ) /AMA-M2 or anti- sp100 antibody and anti- gp210 antibody in serum .

2.

High titer of anti-mitochondrial antibody ( AMA ) /AMA-M2 or anti- sp100 antibody and anti- gp210 antibody in serum .

3.

The histopathology of liver showed changes such as non-suppurative destructive cholangitis and destruction of interlobular bile ducts
.

3.
The histopathology of liver showed changes such as non-suppurative destructive cholangitis and destruction of interlobular bile ducts
.

3.
Treatment of PBC

3.
Treatment of PBC 3.
Treatment of PBC

At present, ursodeoxycholic acid ( UDCA ) is still the main clinical drug treatment for PBC .
For those with poor response to UDCA treatment, few effective drugs are available for second-line treatment, and the prognosis is poor .

At present, ursodeoxycholic acid ( UDCA ) is still the main clinical drug treatment for PBC .
For those with poor response to UDCA treatment, few effective drugs are available for second-line treatment, and the prognosis is poor .

1.
Basic treatment of PBC

1.
Basic treatment of PBC

UDCA is the first-line drug for the treatment of PBC .
Its mechanism of action includes choleretic, cytoprotective, anti-inflammatory, and immune regulation.
It can improve the biochemical indicators of patients, alleviate pathological changes and delay the progression of the disease
.

UDCA is the first-line drug for the treatment of PBC .
Its mechanism of action includes choleretic, cytoprotective, anti-inflammatory, and immune regulation.
It can improve the biochemical indicators of patients, alleviate pathological changes and delay the progression of the disease
.

It is worth noting that the dose selection of UDCA is very important, 13-15 mg/kg is better than the small dose of 5-7 mg/kg , and it is also better than the high-dose of 23-25 ​​mg/kg , if the patient also takes bile such as cholestyramine Acid chelators should be taken 1 hour in advance or 4 hours later, so as not to affect the efficacy of UDCA .

It is worth noting that the dose selection of UDCA is very important, 13-15 mg/kg is better than the small dose of 5-7 mg/kg , and it is also better than the high-dose of 23-25 ​​mg/kg , if the patient also takes bile such as cholestyramine Acid chelators should be taken 1 hour in advance or 4 hours later, so as not to affect the efficacy of UDCA .

No dose adjustment of UDCA is required for PBC patients with abnormal liver function and renal insufficiency .
The main adverse reactions of UDCA include diarrhea, gastrointestinal reactions, skin itching, etc.
, but the incidence is low .
UDCA should be taken for a long time, and discontinuation may lead to a rebound in biochemical indicators or even disease progression .

No dose adjustment of UDCA is required for PBC patients with abnormal liver function and renal insufficiency .
The main adverse reactions of UDCA include diarrhea, gastrointestinal reactions, skin itching, etc.
, but the incidence is low .
UDCA should be taken for a long time, and discontinuation may lead to a rebound in biochemical indicators or even disease progression .

2.
Second-line treatment

2.
Second-line treatment

For PBC patients who do not respond well to UDCA therapy , the only FDA -approved second-line therapy is 6 - ethylchenodeoxycholic acid - obeticholic acid , which is a farnesoid X receptor agonist.
Clinical trials are underway .

For PBC patients who do not respond well to UDCA , the only FDA - approved second-line therapy is 6 - ethylchenodeoxycholic acid - obeticholic acid , which is a farnesoid X receptor agonist.
Clinical trials are underway in China .

In addition, in recent years, it has been found that fibrate lipid-lowering drugs, such as fenofibrate and bezafibrate, can improve the biochemical indicators of PBC patients with poor UDCA treatment response .
Bezafibrate has been tried clinically, but it is necessary to be alert to possible adverse reactions such as elevated transaminases and elevated creatinine .
Since the drug insert has not been updated, PBC is not currently included in the indications, and the official use still needs to wait for the update of the drug insert .

In addition, in recent years, it has been found that fibrate lipid-lowering drugs, such as fenofibrate and bezafibrate, can improve the biochemical indicators of PBC patients with poor UDCA treatment response .
Bezafibrate has been tried clinically, but it is necessary to be alert to possible adverse reactions such as elevated transaminases and elevated creatinine .
Since the drug insert has not been updated, PBC is not currently included in the indications, and the official use still needs to wait for the update of the drug insert .

3.
Symptomatic treatment

3.
Symptomatic treatment

Itching is the most prominent symptom of PBC , and the main drugs for it are cholestyramine and rifampicin
.
Cholestyramine inhibits the reabsorption of bile acids in the intestinal tract, the recommended dose is 4-16 g/d , and the time interval between taking UDCA and other drugs should be at least 4 hours .
For PBC patients with cholestyramine intolerance or poor efficacy , rifampicin can be used as a second-line treatment.
The recommended dose is 150 mg twice a day .
For patients with poor efficacy, the dose can be gradually increased to 600 mg/d .
Liver function should be closely monitored .

Itching is the most prominent symptom of PBC , and the main drugs for it are cholestyramine and rifampicin
.
Cholestyramine inhibits the reabsorption of bile acids in the intestinal tract, the recommended dose is 4-16 g/d , and the time interval between taking UDCA and other drugs should be at least 4 hours .
For PBC patients with cholestyramine intolerance or poor efficacy , rifampicin can be used as a second-line treatment.
The recommended dose is 150 mg twice a day .
For patients with poor efficacy, the dose can be gradually increased to 600 mg/d .
Liver function should be closely monitored .

4.
Liver transplantation

4.
Liver transplantation

Liver transplantation may be considered in patients with refractory ascites, spontaneous peritonitis , recurrent esophagogastric varices bleeding, hepatic encephalopathy, hepatocellular carcinoma and other conditions whose expected survival time is less than 1 year .
The European Society of Liver Diseases recommends that liver transplantation should be evaluated when the total bilirubin level reaches 60 mg/L , the Mayo score reaches 7.
8 , and the end-stage liver disease model score greater than 14 .

Liver transplantation may be considered in patients with refractory ascites, spontaneous peritonitis , recurrent esophagogastric varices bleeding, hepatic encephalopathy, hepatocellular carcinoma and other conditions whose expected survival time is less than 1 year .
The European Society of Liver Diseases recommends that liver transplantation should be evaluated when the total bilirubin level reaches 60 mg/L , the Mayo score reaches 7.
8 , and the end-stage liver disease model score greater than 14 .

4.
Follow-up evaluation and management of PBC

4.
Follow- up evaluation and management of PBC 4.
Follow- up evaluation and management of PBC

PBC is a chronic disease that requires long-term follow-up and cooperation between doctors and patients
.
After the initial diagnosis, UDCA treatment is given, and a follow - up visit is generally recommended after 1 month, and then every 3 months .
After 6-12 months, if it is judged that the patient has a poor response to UDCA treatment, second-line treatment can be added.
After that, it can be re-examined every 1-3 months to evaluate the response of biochemical indicators and the tolerance to the drug, and adjust the treatment.
To achieve biochemical index remission; if the response to UDCA treatment is good, it can be changed to review every 3 to 6 months .

PBC is a chronic disease that requires long-term follow-up and cooperation between doctors and patients
.
After the initial diagnosis, UDCA treatment is given, and a follow - up visit is generally recommended after 1 month, and then every 3 months .
After 6-12 months, if it is judged that the patient has a poor response to UDCA treatment, second-line treatment can be added.
After that, it can be re-examined every 1-3 months to evaluate the response of biochemical indicators and the tolerance to the drug, and adjust the treatment.
To achieve biochemical index remission; if the response to UDCA treatment is good, it can be changed to review every 3 to 6 months .

During the follow-up, in addition to evaluating biochemical indicators, it is also necessary to pay attention to the patient's psychological status, which can be evaluated by scales such as the visual analog scale for itching or fatigue
.
In addition, patients should be regularly checked for liver ultrasound imaging changes, bone mineral density and other osteoporosis-related indicators, blood lipids, assessment of the risk of cardiovascular events, assessment of the risk of liver malignancies such as alpha-fetoprotein, and portal hypertension such as esophageal varices.
The quality of life and disease progression of the patients were comprehensively assessed
.

During the follow-up, in addition to evaluating biochemical indicators, it is also necessary to pay attention to the patient's psychological status, which can be evaluated by scales such as the visual analog scale for itching or fatigue
.
In addition, patients should be regularly checked for liver ultrasound imaging changes, bone mineral density and other osteoporosis-related indicators, blood lipids, assessing the risk of cardiovascular events, assessing the risk of cardiovascular events of liver malignancies such as alpha-fetoprotein, and portal hypertension such as esophageal varices.
The patient's quality of life and disease progression were comprehensively assessed .
Quality of Life

References:

References:

Zhang Fengchun , Wang Li , Shuai Zongwen , et al .
Standard for diagnosis and treatment of primary biliary cholangitis (2021)[J].
Chinese Journal of Internal Medicine , 2021, 60(8):7.

Zhang Fengchun , Wang Li , Shuai Zongwen , et al .
Standard for diagnosis and treatment of primary biliary cholangitis (2021)[J].
Chinese Journal of Internal Medicine , 2021, 60(8):7.
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