If you go to the hospital for a physical examination and find abnormal liver function, or even have developed liver or spleen enlargement, liver fibrosis or cirrhosis, but do not drink alcohol all the time, do not have viral infections (such as hepatitis A, B, C, D, E, etc.), do not take hepatotoxic drugs (such as anti-tuberculosis drugs, certain Chinese medicines, etc.), do not have genetic metabolic diseases (such as hepatomegaly), and do not have fatty liver, then you should be alert to whether you have autoimmune liver disease ( Hereinafter referred to as autoimmune liver disease, you can do the relevant autoantibody test to confirm, or exclude.
What is autoimmune liver: To understand autoimmune liver, first we need to know what immunity is. The immune function is the fundamental guarantee of the organism to protect itself from microbial infections and thus survive. This immune function is carried out by the immune system. Its composition and mechanism are very complicated. In addition to the function of “defending the outside”, it also has the function of “hawking the inside”, that is to say, it should be able to distinguish its own cells that have become bad and remove them. This is the mechanism that keeps people from growing tumors. The immune system is so powerful that it has to be “clean and honest”, and it can’t do anything in its hands, so the immune system must also have its own stable institutions, which should do what it should do and should not do what it should not do. If this self-stabilizing body loses its stability and balance, it will not be able to distinguish between the enemy and the self, and thus can easily harm its own cells and tissues, which seriously leads to “autoimmune disease”.
All autoimmune diseases are the result of immune imbalance. Autoimmune liver, which is the result of immune imbalance, is often accompanied by damage to other systems, organs and tissues, and vice versa, and other autoimmune diseases, such as autoimmune thyroiditis, ulcerative colitis, dry syndrome, rheumatoid arthritis, etc., may also be combined with autoimmune liver.
What are the characteristics of autoimmune liver compared to other hepatitis? It can be said that it is a kind of chronic liver disease, the performance is similar, mainly manifested by abnormal liver function and pathological damage to the liver. However, it is not contagious and does not heal on its own. In the past, it was thought that this disease was mostly seen in white women, but now with the progress of medicine in China, we know that it is not uncommon in Chinese people, of course, more women than men, the ratio is about 9:1, and the number of cases on the outpatient clinic is increasing year by year. At the beginning of the disease, the feeling is not obvious, if not for the unit checkup, are not aware of it, so some people, once discovered is cirrhosis, or even acute and chronic liver failure, this disease is currently no good way at home and abroad, Western medicine that eat ursodeoxycholic acid effective, but this drug can not change the outcome of the disease, can not slow down the development of cirrhosis. For glucocorticosteroids, many experts do not recognize that it may aggravate the patient’s condition, of course, some patients have short-term effects, but the side effects are not small. Liver transplantation may be no solution, but it is expensive, the liver source is not easy to find, and the average family cannot afford it.
Self-exempt liver can be divided into 4 categories: autoimmune hepatitis AIH, primary biliary cirrhosis PBC, primary sclerosing cholangitis PSC and overlap syndrome. The overlap syndrome is any two of AIH, PBC, and PSC at the same time.
There are many similarities in the signs and symptoms of these diseases, starting with the common symptoms of chronic liver disease such as jaundice (yellow skin, sclera, and urine), pruritus, malaise, abdominal and hepatic discomfort, and the common signs of chronic liver disease such as hepatomegaly, spider nevus, and hepatosplenomegaly. This is followed by a series of symptoms of immune diseases such as fever and painful joint soreness.
The disease is prone to develop around the time of menstruation (45-64 years old). The age of onset in men is mostly between 50-70 years old.
1. Symptoms of AIH: Half of the patients with AIH have no symptoms when they start to develop. One-third of patients are already cirrhotic by the time they are found. About half of the patients have other autoimmune diseases, mostly autoimmune thyroiditis. Many patients also have fever and arthralgias, which are similar to those of lupus, so AIH was called “lupus hepatitis” in the 1950s.
2, the cause of AIH: why will get this disease, there are simply three reasons: parents to give, their own chaos, others let you chaos. 1, parents to give that is genetic factors, with a set of genes located on chromosome 6, of course, not parents have the disease, the children will get, but the probability of getting the disease than ordinary people is much greater. 2, their own chaos that is immune factors, the immune system spontaneous destruction of liver cells. 3, others let you mess up that is, environmental factors
The virus (such as hepatitis A and B virus) infects the human body, and liver cells produce the same signal, the immune system in the attack is only recognize the signal, so it is mistaken for the liver cells are viruses, and then launch an attack led to the damage of liver cells.
3, AIH laboratory test, liver function mainly depends on four transaminases and bilirubin, ALT and AST are hepatocyte enzymes, that is to say, these two enzymes will be elevated when the liver cells are damaged, alkaline phosphatase – ALP, transpeptidase – gamma-GGT, are bile duct enzymes, so it will be elevated when the bile duct is damaged. Bilirubin, TBIL/DBIL, is related to jaundice, and the more severe the jaundice, the higher it is.
AIH is hepatocellular damage, so the abnormalities of liver function are mainly elevated ALT and AST, which are about 3-10 times higher than the normal value. Bile duct enzymes and bilirubin are generally normal.
Autoantibodies are proteins produced by the immune system to destroy harmful substances. Autoantibodies attack the cells and tissues of one’s own body and are normally absent or rare in the blood, but if the amount is high, it means that the immune system is disrupted, which means that autoimmune disease has occurred. There are many kinds of autoantibodies, including anti-nuclear antibodies ANA, anti-smooth muscle antibodies SMA, anti-liver and kidney microsomal antibodies
The two types of AIH are distinguished according to the difference of autoantibodies. Type I ANA, SMA positive. It should be noted here that the vast majority of patients with autoimmune diseases and hepatitis C will be positive for ANA, so not all people with positive ANA have self-exempt liver. Eighty percent of autoimmune hepatitis patients are type I. The disease is milder if the patient is an adult. Type II ANA, SMA negative, anti-liver and kidney microsomal antibodies anti-LKM positive, is severe, and children have this type more often.
4. Pathology and diagnosis of AIH: For the diagnosis of AIH, there is a point system, including some laboratory indicators and pathological features, which can be clarified by reaching a certain score. The pathological features are dominated by hepatocellular damage.
5. Treatment of AIH: If you go to a western hospital, usually the doctor will recommend you to use glucocorticoids plus immunosuppressant treatment, but immunosuppressants such as azathioprine itself has hepatotoxicity; hormones are mentioned earlier as possibly aggravating the disease.
Second, primary biliary cirrhosis PBC mostly occurs in middle-aged women, the age of onset is mostly 40-60 years old, with a male to female ratio of 1:9. Although male incidence is less, male PBC patients are more likely to develop hepatocellular carcinoma. It should be noted that the disease starts as an inflammatory disease of the liver, and cirrhosis occurs at a later stage, and is more appropriately called “chronic non-suppurative granulomatous cholangitis”, but the name of the disease has not been corrected due to historical reasons. More than 70% of patients have a combination of dry syndrome (dry mouth, dry eyes, etc.), gallstones and joint disease may also appear at the same time.
1.The etiology of PBC: similar to AIH, it is caused by genetic, immune and environmental factors, but the target of immune system attack becomes intrahepatic middle and small bile ducts.
2.Progression process of PBC: It can be divided into 3 stages. In the first 15-20 years, there are no obvious symptoms, and physical examination may reveal elevated bile duct enzymes or positive autoantibody AMA; after that, 5-10 years, there will be jaundice, weakness and other symptoms, and more particularly, PBC patients will develop skin xantholipoma due to elevated blood lipids. If the disease progresses further, there will be increased jaundice, large belly ascites, vomiting blood, mental confusion and other symptoms of advanced cirrhosis.
3.Laboratory tests and diagnosis of PBC: First read the laboratory tests of AIH to understand the basic content about the self-free liver tests. Liver function: mainly bile duct enzymes are significantly elevated, and ALP is greater than the upper limit of normal by 3-5 times. The bilirubin TBIL is significantly elevated and may exceed 200 mg/L as the disease worsens. hepatocyte enzymes ALT
AST is normal or mildly elevated. Autoantibody test: PBC has special correlation with AMA-M2, if adult is positive for AMA-M2, the diagnosis of PBC can be confirmed basically, no need to do liver puncture. if negative for AMA-M2, liver puncture is needed to confirm the diagnosis, pathological manifestation is inflammation of medium and small bile ducts in liver.
4.Treatment of PBC.
The only effective treatment recognized by FDA is ursodeoxycholic acid capsule, but this drug cannot slow down the progression of cirrhosis. There were several patients with severe PBC who were already in the decompensated stage of cirrhosis, with ascites and vomiting blood, and were at the edge of life and death when they visited the clinic. The director took pity on the patient and treated him with acupuncture twice a week in the outpatient clinic while taking traditional Chinese medicine. One year later, the patient obviously felt a stronger sense of acupuncture, and his energy and strength had improved significantly.
Third, primary sclerosing cholangitis PSC compared with AIH, PBC is more rare, and mostly seen in young men, the ratio of men to women is 2:1, usually heavy disease, in general, the onset of the disease can live 12-17 years, about 10% of patients eventually develop bile duct cancer, about 70% of patients with inflammatory bowel disease, which is also an autoimmune disease, mainly ulcerative colitis, Crohn’s disease, symptoms are abdominal pain and pus and blood stools.
1, the etiology of PSC: mainly genetic and immune factors, immune attack on the intra- and extra-hepatic bile ducts. PBC is a small and medium-sized bile duct lesion, while PSC is mainly a lesion of the bold duct.
2, PSC’s laboratory tests: first read the AIH’s laboratory tests to understand the basic content about self immune liver tests. Liver function: bile duct enzymes ALP is elevated more than 2 times, but some patients have normal ALP, hepatocyte enzymes ALT and AST are mildly elevated (less than 2 times), and bilirubin fluctuates and can be normal or elevated. Unlike AIH and PBC, the probability of autoantibody positivity is not high.
4. Pathology and diagnosis of PSC: endoscopic retrograde cholangiography and magnetic resonance cholangiopancreatography, which is to diagnose by injecting contrast into the bile ducts, this is the gold standard for diagnosing PSC. Pathological examination, bile ducts around a layer of “onion skin” like tissue.
5, treatment: Western medicine currently has no effective treatment drugs. Our department has also used Chinese medicine to treat several cases of such patients, but it seems that the effect is not very obvious, in any case, to further explore.
Overlap syndrome is rare clinically, among which AIH-PBC overlap syndrome, AIH-PSC overlap syndrome is more common, PBC-PSC overlap syndrome is very rare.
The overlap syndrome occurs when the symptoms, laboratory tests and pathology of both diseases are present.
After reading this article, I think you have a more comprehensive understanding of autoimmune liver disease, and I hope it will help you to see the doctor clearly and live happily. If you want to achieve better results, I hope you will read the director’s other articles before you visit him and follow the requirements of the articles strictly.