Autoimmune Hepatitis: Causes, Symptoms, Diagnosis, Treatment

Article by Dr Raghuram Y.S. MD (Ay)
Autoimmune Hepatitis is a chronic disease of the liver caused due to the autoimmune process afflicting the Liver functions. It was previously called by the name ‘Lupoid Hepatitis’. 

Immunity is our in-borne protective mechanism. By any cause if this protective mechanism loses its basic intelligence of protecting our system, turns hostile and starts attacking the body’s own tissues and organs leading to severe damage and causing many stubborn diseases, we call the mechanism as ‘autoimmunity’ or ‘autoimmune process’.

Autoimmune Hepatitis is one such chronic, autoimmune disease of the liver which occurs when the body’s immune system (which has turned hostile) attacks the liver cells causing the liver to be inflamed. (Hepatitis = inflammation of the liver)

liver gall bladder pancreas

liver gall bladder pancreas

Common symptoms of Autoimmune Hepatitis –
Initially the disease is not marked by predominant signs and symptoms specific of the disease. The disease is detected by abnormal liver function tests. The common symptoms of autoimmune hepatitis are –

  • Fatigue
  • Muscle pains
  • Fever
  • Jaundice
  • Pain abdomen, concentrated mainly in the right upper quadrant

How Autoimmune Hepatitis is caused?
The disease occurs on the backdrop of genetic predisposition or acute liver infection. Due to these causes, there occurs an anomalous presentation of HLA (Human Leukocyte Antigen) class II on the surface of liver cells. This causes a cell mediated immune response against the body’s own liver. This in turn leads to inflammation of the Liver. This condition is called Autoimmune Hepatitis. This abnormal immune response results in liver inflammation. This can lead to further symptoms and complications including fatigue, cirrhosis of liver etc. it may occur at any age and in any ethnic group. It is most often diagnosed in patients belonging to the age group between 40 and 50 years.

Cause of Autoimmune Hepatitis
60% patients have chronic hepatitis that may mimic viral hepatitis, but without evidence of viral infection
The disease usually affects women
It is strongly associated with anti-smooth muscle auto-antibodies

Signs and Symptoms of Autoimmune Hepatitis
The disease presents with signs of chronic liver disease like fatigue, body aches and abnormal liver function tests. It also comprises of signs and symptoms of acute hepatitis like fever, jaundice and pain in the right upper quadrant of the abdomen
It occurs in women (70% cases) between the ages 15 and 40 years.

Lupoid Hepatitis was the name with which this disease was called earlier. But the fact is that both autoimmune hepatitis and Systemic Lupus Erythmatosus (SLE) are 2 distinct diseases. People with SLE do not have an increased incidence of autoimmune hepatitis.

Evidence of moderate to severe hepatitis with elevated serum ALT and AST activities along with normal to marginal elevation of ALP (Alkaline phosphatise) and gamma glutamyl transpeptidase activities

Occasional systemic symptoms –

  • Arthralgia – Joint pains usually mild and intermittent, predominantly present in the morning
  • Myalgia
  • General feeling of ill health
  • Lack of energy
  • Easily getting tired
  • Inability to finish a full day work
  • Tendency to sleep more
  • Polyserositis
  • Thrombocytopenia
  • Mild / Severe hepatic dysfunction

Less common symptoms –

  • Nausea
  • Decreased appetite
  • Weight loss
  • Abdominal pain and bloating
  • Indigestion
  • Jaundice
  • Abnormal blood vessels on the skin on the face, arms and chest
  • Bruising

Prevention of Autoimmune Hepatitis
The cause of AIH as yet remains unknown. You will not develop AIH by exposure to someone else with the disease. It is not thought to be due to a virus and has nothing to do with alcohol. Although there is a genetic link to AIH, at the present time there is nothing that can be done to prevent the condition occurring.

No role of diet in preventing AIH – Researchers have not found that eating, diet and nutrition play a role in causing or preventing autoimmune hepatitis.

Autoimmune Hepatitis is an autoimmune disorder. Therefore it cannot be prevented in most cases. Knowing the risk factors may help you to detect and treat the disease early.

Precautions can be taken to see that the disease doesn’t progress and to see that the liver health is optimum and good. The following may be followed –

Physical examination and blood tests once in every few months
Liver biopsy repeated at least once in 2 years
Self care – Taking medication and seeing healthcare provider on regular basis
Diet – No specific diet has been shown to improve the outcome in AIH. The best advice is to eat normal, healthy and balanced diet and to avoid becoming obese. Obesity can increase the risk of fatty liver disease and may complicate autoimmune hepatitis.

Alcohol – should be avoided since it can cause fatty liver and other liver damage. All types of alcoholic beverages can be harmful to the liver. Patients with liver disease may worsen with even small amounts of alcohol.
Exercise – is good for overall health and is highly recommended. Of course, it doesn’t have specific benefit or disease modifying role.

Be careful with the medications – Most medicines and drugs are broken down in the liver. It is better to check with a healthcare provider or pharmacist before starting a new prescription. Unless the liver is damaged or scarred, the drugs are safe. Some people with active liver disease will be advised to take a smaller dose of medication.

Prednisone – Standard treatments like prednisone have been proven to delay or prevent progression to cirrhosis and thus save lives.
Read related: Autoimmune Hepatitis Ayurvedic Concept, Explanation, Treatment

Giving favourable working conditions for liver – Favour your liver and enhance its health by eating healthy well-balanced diet, not smoking and drinking alcohol only in modest amounts or abstaining altogether. If you are overweight, look for a gradual and sustained weight loss. Introduce exercise into your daily routine; include walking, swimming, gardening, stretching etc. Healthy diet and exercise are important components of weight-loss programme.

Reduce calorie rich foods such as sugar, sweets, cakes, biscuits, fried food, pasties and pies, crisps and chocolate. Use low-fat versions instead and fill up on fruit and vegetables.

Diagnosis –
Clinical, laboratory and histological findings all put together serve in the diagnosis of autoimmune hepatitis. Before making diagnosis of this condition, the other etiological factors (viral, hereditary, metabolic, cholestatic and drug-induced diseases of liver) should be ruled out

Diagnosis of autoimmune hepatitis is clinched with the presence of the below mentioned antibodies in the blood –

  • ANA (anti nuclear antibody)
  • SMA (anti-smooth muscle antibody)
  • LKM (Liver Kidney Microsomal Antibody) – LKM-1, LKM-2, LKM-3
  • SLA/LP (anti soluble liver antigen and liver-pancreas antigen)
  • AMA (anti-mitochondrial antibody)

Similarly demonstration of increased immunoglobulin G (IgG) level in the blood is needed for diagnosis to be made
Diagnosis of Autoimmune Hepatitis always requires a Liver Biopsy.
Primary biliary cirrhosis and primary sclerosing cholangitis have been observed to overlap the presentation of autoimmune hepatitis. More on diagnosis

Differential Diagnosis of Autoimmune Hepatitis –
Hepatitis A – It is a highly contagious liver infection caused by Hepatitis A virus. It can cause mild to severe illness. HAV (Hepatitis A Virus) is transmitted through ingestion of contaminated food and water or through direct contact with an infectious person. In this, you will have inflammation of the liver caused by the virus. Symptoms if any include jaundice, pain in the belly, loss of appetite, nausea, fever, diarrhoea, fatigue etc.

Hepatitis B – It is a viral infection that attacks the liver and can cause both acute and chronic disease. It can cause chronic infection and puts people at high risk of death from cirrhosis and liver cancer. The vaccine against Hepatitis B is 95% effective in preventing infection. It is most commonly spread by exposure to infected body fluids, blood products (unclean needles or unscreened blood), and mother to baby and unprotected vaginal, anal or oral sex. Symptoms are variable and include yellowing of the eyes, abdominal pain and dark urine. In chronic cases, liver failure, or scarring can occur. Chronic cases need medication and possibly liver transplant.

Hepatitis C – It is an infectious disease of the Liver caused by Hepatitis C Virus (HCV). The virus can cause both acute and chronic hepatitis infection, ranging in severity from a mild illness lasting few weeks to a serious lifelong illness. The virus spreads by contact with contaminated blood, for example, from sharing needles or from unsterile tattoo equipments and by mother to baby by pregnancy, labour or nursing. Most people have no symptoms. Those who develop symptoms have fatigue, nausea, loss of appetite and yellowing of the skin and eyes.

Hepatitis D – Hepatitis D is caused by Hepatitis D Virus (HDV), also called Delta virus. It is an infection that causes the liver to become inflamed. This swelling can impair liver function and cause long term liver problems, including liver scarring and cancer. It occurs only among people who are infected with Hepatitis B Virus. Transmission requires contact with infectious blood, also spreads from mother to child. Symptoms include abdominal pain, nausea and fatigue.

Hepatitis E – Hepatitis E is a liver disease caused by infection with a virus called Hepatitis E Virus (HEV). It is usually self-limiting but some cases may develop into fulminant hepatitis (acute liver failure). It is mainly transmitted through drinking water contaminated with faecal matter. Symptoms include jaundice, lack of appetite and nausea. In rare cases, it may progress to acute liver failure. It resolves on its own within 4-6 weeks.

Primary Biliary Cirrhosis – It is also known as Primary Biliary Cholangitis (PBC). It is an autoimmune disease of the liver. It is marked by slow progressive destruction of the small bile ducts of the liver, with the intralobular ducts and the Canals of Hering (intrahepatic ductules) affected early in the disease. It may lead to progressive cholestasis and often end stage liver disease.

Primary Sclerosing Cholangitis – It is a chronic liver disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. It is a disease of the bile ducts. It is strongly associated with inflammatory bowel disease. The disease may progress slowly and can lead to liver failure, repeated infections, and tumours of the bile duct or liver.

Genetic Haemochromatosis – It is also called Hereditary Haemochromatosis (HHC). It is a heterogenous group of disorders related to deficiency of the iron regulatory hormone hepcidin. HHC is an autosomal recessive genetic disease in which increased intestinal absorption of iron causes accumulation in tissues, especially the liver, which may lead to organ damage. Patients can present with cirrhosis, polyarthropathy, adrenal insufficiency, heart failure or diabetes, fatigue, malaise etc symptoms.

Wilson Disease – It is a genetic (autosomal recessive inherited) disorder of copper metabolism that is characterized by excessive deposition of copper in the liver, brain and other tissues. It is often fatal if not recognized and treated when symptomatic. The liver related symptoms include vomiting, weakness, fluid build up in the abdomen, swelling of the legs, yellowish skin and itching.

Non-alcoholic steatohepatitis (Fatty Liver Disease) – It is a common, often silent liver disease. It resembles alcoholic liver disease, but occurs in people who drink little or no alcohol. It is liver inflammation and damage caused by a build up of fat in the liver. It can get worse over a period of time and cause scarring of the liver, which leads to cirrhosis. It also causes liver cell injury and steatosis.

Alcoholic Liver Disease – It is the most advanced form of liver disease that is related to drinking alcohol. It is a term which encompasses the liver manifestations of alcohol overconsumption, including fatty liver, alcoholic hepatitis and chronic hepatitis with liver fibrosis or cirrhosis.

Systemic Lupus Erythmatosus – It is a chronic and often disabling auto-immune disease. Lupus can affect the joints, skin, kidneys, blood cells, brain, heart and lungs. Symptoms include fatigue, joint pain, rash and fever.

Granulomatous Hepatitis – It is a multi-factorial liver disorder with or without additional hepatic inflammation and fibrosis. Hepatic granulomas may be accompanied by severe inflammation within or surrounding granulomatous structures which is termed as granulomatous hepatitis. Infectious diseases are the most common causes. Symptoms include minor hepatomegaly and little or no jaundice, fever, malaise etc.

Cirrhosis of Liver – It is the severe scarring of the Liver and poor liver function seen at the terminal stages of chronic liver disease. Hepatitis and chronic alcohol abuse are frequent causes. Liver damage caused by cirrhosis cannot be reversed or undone, but further damage can be limited. Symptoms include fatigue, weakness and weight loss, yellowing of skin (jaundice), gastrointestinal bleeding, abdominal swelling and confusion.

Classification of Autoimmune Hepatitis
Below mentioned are the 4 sub-types of Autoimmune Hepatitis –
Positive ANA and SMA, elevated immunoglobulin G (classic form, responds well to low dose of steroids)
Positive LKM-1 (typically female children, teenagers, disease can be severe), LKM-2 or LKM-3
Positive antibodies against soluble liver antigen, same as group 1 (anti-SLA, anti-LP)
No auto-antibodies detected (debatable validity / importance, less than 20%)

Treatment
Immune-suppressive gluco-corticoids such as prednisolone (with or without azathioprine), chances of relapse of symptoms are more in spite of treatment
Budesonide – more effective than prednisolone in inducing remission, few adverse results
Other immunosuppressive drugs – mycophenolate, ciclosporin, tacrolimus, methotrexate etc
Liver transplantation (when drug therapy fails, or when patients present with fulminant liver failure)

Prognosis

  • Autoimmune hepatitis is not a benign disease
  • The disease responds well to immunosuppression initially
  • Recent studies show that the life expectancy of patients with autoimmune hepatitis is lower than that of the general population
  • Presentation of the disease and response to the therapy is found to differ according to race
  • African Americans appear to present with a more aggressive disease associated with worse outcomes

Epidemiology
Autoimmune Hepatitis has an incidence of 1-2 per 100.000 per year and a prevalence of 10-20 / 100,000.
It affects women much more than men (70%)
Liver enzymes and bilirubin are elevated (Source)

History
Autoimmune Hepatitis was previously called ‘Lupoid Hepatitis’ and was linked to ‘Systemic Lupus Erythmatosus’. This condition was originally described in the early 1950s.

Most patients do have an associated autoimmune disorder such as systemic lupus erythmatosus, therefore it was called lupoid hepatitis
The term lupoid hepatitis is no longer used because autoimmune hepatitis has multiple different presentations (forms) and is not always associated with SLE. The current name at present is Autoimmune Hepatitis (AIH)
Click to consult Dr Raghuram Y.S. MD(Ayu)


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