Lupoid hepatitis
Encyclopedia
Lupoid hepatitis is an auto-immune disease which causes liver cirrhosis
. It maybe associated with systemic lupus erythematosus
(SLE) or other connective tissue disorders. 60% of patients have chronic hepatitis
that may mimic viral hepatitis, but without serologic
evidence of a viral infection. The disease usually affects women and is strongly associated with anti-smooth muscle
auto-antibodies
.
Autoimmune hepatitis is a condition in which the patient's own immune systems attacks the liver causing inflammation and liver cell death. The condition is chronic and progressive. Although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis.
"Autoimmune hepatitis usually occurs in women (70 %) between the ages of 15 and 40. Although the term "lupoid" hepatitis was originally used to describe this disease, patients with systemic lupus erythematosus do not have an increased incidence of autoimmune hepatitis and the two diseases are distinct entities. Patients usually present with evidence of moderate to severe hepatitis with elevated serum ALT and AST activities in the setting of normal to marginally elevated alkaline phosphatase and gamma-glutamyltranspeptidase activities. The patient will sometimes present with jaundice, fever and right upper quadrant pain and occasionally systemic symptoms such as arthralgias, myalgias, polyserositis and thrombocytopenia. Some patients will present with mild liver dysfunction and have only laboratory abnormalities as their initial presentation. Others will present with severe hepatic dysfunction."
Autoimmune hepatitis is a chronic inflammatory disease of the liver. Amino transferases are elevated in patients who have had viral metabolic and toxic events ruled out.
Autoimmune hepatitis was previously called "lupoid" hepatitis. It was originally described in the early 1950s.
Most patients do have an associated autoimmune disorder such as systemic lupus erythematosis. Thus, its name was previously lupoid hepatitis.
Because the disease has multiple different forms, and is not always associated with systemic lupus erythematosis, lupoid hepatitis is no longer used. The current name at present is autoimmune hepatitis (IAH).
Autoimmune disorders are divided according to the type of antibody that is present. In general, there are four major classifications. These are ANA ( anti-nuclear antigen), SMA (smooth muscle antigen), and anti LKM (anti liver kidney miscrosomal) positive subtypes.
Evaluation for these types of autoimmune hepatitis is obtained through simple blood tests.
Based upon the serology and associated rheumatologic disorders, a patient may be treated with prednisone
, etc.
In general, patients do quite well with autoimmune hepatitis. Treatment is usually required several years in order to bring the liver function tests, hepatic inflammation and changes into more normal perimeters.
Most of the improvement in liver function tests occurs within the first 6 months of treatment. However, changes in the liver histology do require longer-term therapy. This usually occurs 6–12 months after liver function tests have improved. Thus, long-term treatment is required for good control of autoimmune hepatitis patients.
Cirrhosis
Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrosis, scar tissue and regenerative nodules , leading to loss of liver function...
. It maybe associated with systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
(SLE) or other connective tissue disorders. 60% of patients have chronic hepatitis
Hepatitis
Hepatitis is a medical condition defined by the inflammation of the liver and characterized by the presence of inflammatory cells in the tissue of the organ. The name is from the Greek hepar , the root being hepat- , meaning liver, and suffix -itis, meaning "inflammation"...
that may mimic viral hepatitis, but without serologic
Serology
Serology is the scientific study of blood serum and other bodily fluids. In practice, the term usually refers to the diagnostic identification of antibodies in the serum...
evidence of a viral infection. The disease usually affects women and is strongly associated with anti-smooth muscle
Smooth muscle
Smooth muscle is an involuntary non-striated muscle. It is divided into two sub-groups; the single-unit and multiunit smooth muscle. Within single-unit smooth muscle tissues, the autonomic nervous system innervates a single cell within a sheet or bundle and the action potential is propagated by...
auto-antibodies
Antibody
An antibody, also known as an immunoglobulin, is a large Y-shaped protein used by the immune system to identify and neutralize foreign objects such as bacteria and viruses. The antibody recognizes a unique part of the foreign target, termed an antigen...
.
Autoimmune hepatitis is a condition in which the patient's own immune systems attacks the liver causing inflammation and liver cell death. The condition is chronic and progressive. Although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis.
"Autoimmune hepatitis usually occurs in women (70 %) between the ages of 15 and 40. Although the term "lupoid" hepatitis was originally used to describe this disease, patients with systemic lupus erythematosus do not have an increased incidence of autoimmune hepatitis and the two diseases are distinct entities. Patients usually present with evidence of moderate to severe hepatitis with elevated serum ALT and AST activities in the setting of normal to marginally elevated alkaline phosphatase and gamma-glutamyltranspeptidase activities. The patient will sometimes present with jaundice, fever and right upper quadrant pain and occasionally systemic symptoms such as arthralgias, myalgias, polyserositis and thrombocytopenia. Some patients will present with mild liver dysfunction and have only laboratory abnormalities as their initial presentation. Others will present with severe hepatic dysfunction."
Autoimmune hepatitis is a chronic inflammatory disease of the liver. Amino transferases are elevated in patients who have had viral metabolic and toxic events ruled out.
Autoimmune hepatitis was previously called "lupoid" hepatitis. It was originally described in the early 1950s.
Most patients do have an associated autoimmune disorder such as systemic lupus erythematosis. Thus, its name was previously lupoid hepatitis.
Because the disease has multiple different forms, and is not always associated with systemic lupus erythematosis, lupoid hepatitis is no longer used. The current name at present is autoimmune hepatitis (IAH).
Diagnosis
Patients who have chronic elevations in liver function tests for greater than 6 months are thought to have chronic hepatitis. In these individuals, other diseases are evaluated. When Wilson's disease, hemochromatosis, alpha 1 antitrypsin deficiency, Hepatitis B and C and drug induced hepatitis have been ruled out, a possible autoimmune disorder is present.Autoimmune disorders are divided according to the type of antibody that is present. In general, there are four major classifications. These are ANA ( anti-nuclear antigen), SMA (smooth muscle antigen), and anti LKM (anti liver kidney miscrosomal) positive subtypes.
Evaluation for these types of autoimmune hepatitis is obtained through simple blood tests.
Based upon the serology and associated rheumatologic disorders, a patient may be treated with prednisone
Prednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
, etc.
Treatment
Not all patients with autoimmune hepatitis require treatment. However, when they have serum amino transferase elevations greater than 10 times above normal, scarring of the liver, and/or other features associated with hepatic failure, treatment is indicated. Usually, patients are treated with Prednisone 40 mg po qd. Current therapy may include 6 mercaptopurine with or without steroids.In general, patients do quite well with autoimmune hepatitis. Treatment is usually required several years in order to bring the liver function tests, hepatic inflammation and changes into more normal perimeters.
Most of the improvement in liver function tests occurs within the first 6 months of treatment. However, changes in the liver histology do require longer-term therapy. This usually occurs 6–12 months after liver function tests have improved. Thus, long-term treatment is required for good control of autoimmune hepatitis patients.