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Ms. Wang (pseudonym), a 40-year-old white-collar worker in Hangzhou, never imagined that the itchy skin that had plagued her for more than a year was not because of "dryness", but because of "liver".
Scratches on the arms
One investigation turned out to be autoimmune liver disease
Near the end of the clinic, a lady with exquisite makeup hurriedly walked into the consultation room of Pi Borui, deputy chief physician of the Department of Liver Disease and Infection of Zhejiang University Shaw Hospital, and as soon as she sat down, she said: "Doctor, my skin has been itchy for more than 1 year, and I have been in the dermatology department for a year. Today in another hospital to check, said that it is liver disease, let me quickly hang up a number to come here to see, but also said that may be hospitalized, I am scared to death. ”
As she spoke, Ms. Wang raised her sleeves, revealing scratches on her arms, old and new.
Under the inquiry, Ms. Wang revealed that she has not had a physical examination for a long time, in addition to frequent skin itching, there is nothing uncomfortable, and she will feel tired, but she always thinks that she is too busy at work and has not slept well.
Dr. Pi found Ms. Wang's medical records in a skin hospital in zhejiang through the mutual recognition platform of zhejiang medical examination results, the blood routine prompted normal, the biochemical examination liver function was abnormal, the alanine aminotransferase ALT was slightly elevated, and the alkaline phosphatase (ALP) increased significantly, with more than 400U/L, and no imaging examination data.
Combined with the patient's self-report and the results of the external hospital examination, Dr. Dermatologist suspected that Ms. Wang was immune to liver disease.
On further examination, it is true that there is primary biliary cholangitis (PBC). This is an autoimmune liver disease, which is the cause of Ms. Wang's itchy skin.
When Ms. Wang heard this, she panicked and cried and asked, "Doctor, am I very serious?" Is it time to change the liver? ”
Doctor Pi quickly reassured her: "Don't be afraid, you can cure it!" ”
What are Autoimmune Liver Disease and PBC?
Immune systems are like our body's police, they defend our body's health through immune defense, immune self-stabilization, and immune surveillance.
Under normal circumstances, the immune system can distinguish between "foreign invaders" (such as bacterial viruses) and "own people" (such as various tissues and organs of the body), maintaining the stability of the internal environment of the body.
If the immune system goes wrong, there will be a situation that harms "one's own people". Among them, when the liver and bile duct system are "accidentally injured" by the immune system, lesions of the hepatobiliary system will occur, which we call autoimmune liver disease.
Autoimmune liver disease is a group of diseases, and primary biliary cholangitis (PBC) is one of them.
PBC was first named "primary biliary cirrhosis" because patients had entered the cirrhosis phase when they were diagnosed. Now, with the promotion of detection methods and the application of drugs, more and more patients can be diagnosed and treated in the early stages of the disease. In order to alleviate the patient's concerns and distress about the word "cirrhosis" in the name, it was renamed "primary biliary cholangitis" in 2015, and the English abbreviation is PBC.
What are the dangers of PBC?
PBC is a chronic, progressive, destructive cholangitis that progresses to liver fibrosis, cirrhosis, and chronic liver failure. But if detected early and treated early, patients can be no different from other healthy people.
Who is susceptible to PBC?
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PBC is not uncommon, but it has not attracted enough attention from doctors and patients. The data show that the age of onset of PBC is between 20 and 90, the average age is 50 years old, mainly in middle-aged women aged 40 to 60. Worldwide, at least 100,000 people are diagnosed with PBC each year, and the prevalence in women over the age of 40 is 1 in 1,000.
What are the symptoms of PBC?
In the early stages of PBC, most of them have no obvious symptoms of discomfort and are mainly detected by physical examination. As the disease progresses, patients experience fatigue and itchy skin. If you progress to the stage of cirrhosis, you may have symptoms such as jaundice, ascites, lower extremity edema, and gastrointestinal bleeding.
The following methods are commonly used to diagnose PBC
Liver function
Patients with PBC usually present with significantly elevated alkaline phosphatase (ALP) and glutamyl transpeptidase (GGT), while alanine aminotransferase (ALT) and aspartate aminotransferase (AST) are mildly elevated or normal.
Own antibody
Serum AMA is a specific indicator for the diagnosis of PBC, and the positive rate of AMA in patients with PBC can reach 95%. Other antibodies such as anti-gp210 antibodies and anti-sp100 antibodies are also specific antibodies for PBC.
Imaging studies
Includes hepatobiliary ultrasound, CT, and MR. The purpose of these tests is to identify liver function caused by biliary stones or tumors, and secondly, PBC can cause cirrhosis and liver cancer, and we need imaging tests to detect cirrhosis and liver cancer in time.
Liver biopsy
In some cases, doctors may recommend a liver aspiration biopsy to aid in the diagnosis. Such as suspected AMA-negative PBC, or suspected cause of other liver damage.
How is PBC treated? How effective is it?
Preferred
drugs
Ursodeoxycholic acid
Ursodeoxycholic acid (UDCA) is the drug of choice for PBC treatment, which can significantly improve the natural progression of PBC to cirrhosis and chronic liver failure, with few adverse reactions and high safety.
Dermatologists remind that after taking UDCA, some patients have improved liver function, so they stop the drug or reduce the dose on their own, resulting in rebound of liver function and disease progression.
Therefore, patients with PBC need to pay attention to three points:
● Long-term adherence to UDCA, do not easily stop the drug;
● UDCA should be given a therapeutic dose according to body weight, do not easily reduce the dose;
●Regular review to assess the efficacy of UDCA in a timely manner.
Second
Obequicholic acid, fibrates, budesonide
For patients with poor UDCA treatment, second-line drugs can also be combined.
●Obecholic acid: Multiple clinical trials have confirmed that in patients with poor UDCAs, the combination of obecholic acid significantly reduces ALP and improves liver function. The main side effects of obecholic acid are skin itching and lowering high-density lipoprotein cholesterol (HDL). In 2016, Obecholic acid was approved for listing in the United States, Europe and other countries. It is not yet listed on the mainland, and phase III clinical trials of Oberbicholic acid are being carried out, and Zhejiang University Shaw Hospital is one of the research units.
●Fibrates: are drugs used to treat hyperlipidemia, such as fenofibrate and benzafibrate. The main adverse effects of fibrates are liver function impairment and muscle damage, and liver function and creatine kinase monitoring are required during treatment.
●Budesonide: a second-generation corticosteroid, clinical studies have shown that budesonide can improve liver function and disease progression in patients with early PBC, but there are more side effects in advanced patients, so it is not recommended for patients with cirrhosis. At present, budesonide oral preparations have not been listed in Chinese mainland.
Liver transplantation
The only effective treatment for terminally ill patients
Liver transplantation may be considered in patients with cirrhosis PBC: refractory ascites, spontaneous peritonitis, recurrent esophageal gastric variceal rupture and bleeding, hepatic encephalopathy, liver cancer, etc.
The Hepatology and Infection Department team of Zhejiang University Shaw Hospital has extensive experience in the diagnosis and treatment of PBC, especially for refractory PBC (poor UDCA response), PBC overlapping AIH, and cirrhotic PBC. At present, Run Run Shaw Hospital is conducting a phase III clinical trial of Obecholic Acid in patients with poor UDCA responses to promote the early listing of Obequicholic Acid in mainland China and benefit more PBC patients in mainland China.
The study was approved by the State Drug Administration (Clinical Trial Approval Notice Acceptance Number: CYHS2000282) and has passed the review of the Hospital Ethics Committee.
Patients who meet the following basic criteria may be considered for clinical trials:
Patients who are eligible for the diagnosis of PBC;
Age 18 to 75 years (including border values), male or female;
Volunteer to participate in the study, sign an informed consent form, have good compliance, and be able to cooperate with experimental observation.
If you meet the above basic conditions and are willing to participate in this clinical study, please contact Assistant Ma, the chief physician of the Department of Liver Disease and Infection of Zhejiang University Shaw Hospital.
Contact: 18334310408
bibliography:
1． Guidelines for the diagnosis and treatment of primary biliary cholangitis (2021)[J].Journal of Clinical Hepatobiliary Diseases,2022,38(01):35-41.
2． Diagnosis and treatment status and challenges of primary biliary cholangitis[J].Journal of Clinical Hepatobiliary Diseases,2021,37(10):2257-2261.
Source: Run Run Shaw Hospital
Author: Department of Liver Disease Infection Pi Bo Rui
Original works, reprinted please indicate the source and source
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