Auto Immune by Dr. Worman world reknown..... biopsy YES

[Guest guest]

Autoimmune Hepatitis

By J. Worman, M. D.

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 distint 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,

polyserositits 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 should be suspected in any young patient with

hepatitis, especially those without risk factors for alcoholic, drug,

metabolic or viral etiologies. Serum protein electrophoresis and testing

for autoantibodies are of central importance in the diagnosis of

autoimmune hepatitis. Patients with one subtype of autoimmune hepatitis

have serum gamma-globulin concentrations more than twice normal and

sometimes antinuclear antibodies and/or anti-smooth muscle (anti-actin)

antibodies. Patients with another subtype may have normal or only slightly

elevated serum gamma-globulin concentrations but will have antibodies

against a particular cytochrome p450 isoenzyme that are called anti-LKM

(liver kidney microsome).

Patients in whom a diagnosis of autoimmune hepatitis is suspected should

have a liver biopsy. If the biopsy is consistent, treatment with steroids

(prednisone or pednisolone) and azathioprine (Imuran) is begun

immediately. These are tapered over the next 6 to 24 months depending upon

the patient's course. If immediate liver biopsy is contraindicated because

of a prolonged prothrombin time or thrombocytopenia, steroids and

azathioprine should be started prior to biopsy if the diagnosis of

autoimmune hepatitis is likely based on clinical criteria (e.g. a young

woman with severe hepatitis, elevated serum gamma-globulin concentration,

negative risk factors and serologies for viral hepatitis). The patient

will often rapidly improve and biopsy should be performed to confirm the

diagnosis as soon as the prothrombin time decreases and platelet count

increases to within safe ranges.

About two thirds to three quarters of patients with autoimmune hepatitis

respond to treatment based on the return of serum ALT and AST activities

to normal and an improved biopsy after several months. Some patients

relapse as steroids and azathioprine doses are tapered or stopped and need

chronic maintenance medications. Over the long term, many patients develop

cirrhosis despite having a response to treatment, and patients who do not

respond to treatment will almost always progress to cirrhosis. If

end-stage liver disease develops, orthotopic liver transplantation is an

effective procedure.

For more information, you may want to see the following review articles:

Czaja, A.J. and Freese, D. K. 2002. Diagnosis and treatment of autoimmune

hepatitis. Hepatology. 36:479-497.

Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of

Medicine. 334:897-903.

Click here to return to Diseases of the Liver home page.

Click here to go to COLUMBIA UNIVERSITY GASTROENTEROLOGY WEB.

Copyright, 1995, 1996, 1998, 2002, J. Worman, M. D.

Autoimmune Hepatitis/ J. Worman, M. D./hjw14@...

" and the beat goes on....... " Sonny Bono " It's not the years in your life that

count. It's the life in your years. " Abraham Lincoln

__________________________________________________

[Guest guest]

Autoimmune Hepatitis

By J. Worman, M. D.

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 distint 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,

polyserositits 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 should be suspected in any young patient with

hepatitis, especially those without risk factors for alcoholic, drug,

metabolic or viral etiologies. Serum protein electrophoresis and testing

for autoantibodies are of central importance in the diagnosis of

autoimmune hepatitis. Patients with one subtype of autoimmune hepatitis

have serum gamma-globulin concentrations more than twice normal and

sometimes antinuclear antibodies and/or anti-smooth muscle (anti-actin)

antibodies. Patients with another subtype may have normal or only slightly

elevated serum gamma-globulin concentrations but will have antibodies

against a particular cytochrome p450 isoenzyme that are called anti-LKM

(liver kidney microsome).

Patients in whom a diagnosis of autoimmune hepatitis is suspected should

have a liver biopsy. If the biopsy is consistent, treatment with steroids

(prednisone or pednisolone) and azathioprine (Imuran) is begun

immediately. These are tapered over the next 6 to 24 months depending upon

the patient's course. If immediate liver biopsy is contraindicated because

of a prolonged prothrombin time or thrombocytopenia, steroids and

azathioprine should be started prior to biopsy if the diagnosis of

autoimmune hepatitis is likely based on clinical criteria (e.g. a young

woman with severe hepatitis, elevated serum gamma-globulin concentration,

negative risk factors and serologies for viral hepatitis). The patient

will often rapidly improve and biopsy should be performed to confirm the

diagnosis as soon as the prothrombin time decreases and platelet count

increases to within safe ranges.

About two thirds to three quarters of patients with autoimmune hepatitis

respond to treatment based on the return of serum ALT and AST activities

to normal and an improved biopsy after several months. Some patients

relapse as steroids and azathioprine doses are tapered or stopped and need

chronic maintenance medications. Over the long term, many patients develop

cirrhosis despite having a response to treatment, and patients who do not

respond to treatment will almost always progress to cirrhosis. If

end-stage liver disease develops, orthotopic liver transplantation is an

effective procedure.

For more information, you may want to see the following review articles:

Czaja, A.J. and Freese, D. K. 2002. Diagnosis and treatment of autoimmune

hepatitis. Hepatology. 36:479-497.

Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of

Medicine. 334:897-903.

Click here to return to Diseases of the Liver home page.

Click here to go to COLUMBIA UNIVERSITY GASTROENTEROLOGY WEB.

Copyright, 1995, 1996, 1998, 2002, J. Worman, M. D.

Autoimmune Hepatitis/ J. Worman, M. D./hjw14@...

" and the beat goes on....... " Sonny Bono " It's not the years in your life that

count. It's the life in your years. " Abraham Lincoln

__________________________________________________

[Guest guest]

Autoimmune Hepatitis

By J. Worman, M. D.

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 distint 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,

polyserositits 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 should be suspected in any young patient with

hepatitis, especially those without risk factors for alcoholic, drug,

metabolic or viral etiologies. Serum protein electrophoresis and testing

for autoantibodies are of central importance in the diagnosis of

autoimmune hepatitis. Patients with one subtype of autoimmune hepatitis

have serum gamma-globulin concentrations more than twice normal and

sometimes antinuclear antibodies and/or anti-smooth muscle (anti-actin)

antibodies. Patients with another subtype may have normal or only slightly

elevated serum gamma-globulin concentrations but will have antibodies

against a particular cytochrome p450 isoenzyme that are called anti-LKM

(liver kidney microsome).

Patients in whom a diagnosis of autoimmune hepatitis is suspected should

have a liver biopsy. If the biopsy is consistent, treatment with steroids

(prednisone or pednisolone) and azathioprine (Imuran) is begun

immediately. These are tapered over the next 6 to 24 months depending upon

the patient's course. If immediate liver biopsy is contraindicated because

of a prolonged prothrombin time or thrombocytopenia, steroids and

azathioprine should be started prior to biopsy if the diagnosis of

autoimmune hepatitis is likely based on clinical criteria (e.g. a young

woman with severe hepatitis, elevated serum gamma-globulin concentration,

negative risk factors and serologies for viral hepatitis). The patient

will often rapidly improve and biopsy should be performed to confirm the

diagnosis as soon as the prothrombin time decreases and platelet count

increases to within safe ranges.

About two thirds to three quarters of patients with autoimmune hepatitis

respond to treatment based on the return of serum ALT and AST activities

to normal and an improved biopsy after several months. Some patients

relapse as steroids and azathioprine doses are tapered or stopped and need

chronic maintenance medications. Over the long term, many patients develop

cirrhosis despite having a response to treatment, and patients who do not

respond to treatment will almost always progress to cirrhosis. If

end-stage liver disease develops, orthotopic liver transplantation is an

effective procedure.

For more information, you may want to see the following review articles:

Czaja, A.J. and Freese, D. K. 2002. Diagnosis and treatment of autoimmune

hepatitis. Hepatology. 36:479-497.

Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of

Medicine. 334:897-903.

Click here to return to Diseases of the Liver home page.

Click here to go to COLUMBIA UNIVERSITY GASTROENTEROLOGY WEB.

Copyright, 1995, 1996, 1998, 2002, J. Worman, M. D.

Autoimmune Hepatitis/ J. Worman, M. D./hjw14@...

" and the beat goes on....... " Sonny Bono " It's not the years in your life that

count. It's the life in your years. " Abraham Lincoln

__________________________________________________

[Guest guest]

Autoimmune Hepatitis

By J. Worman, M. D.

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 distint 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,

polyserositits 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 should be suspected in any young patient with

hepatitis, especially those without risk factors for alcoholic, drug,

metabolic or viral etiologies. Serum protein electrophoresis and testing

for autoantibodies are of central importance in the diagnosis of

autoimmune hepatitis. Patients with one subtype of autoimmune hepatitis

have serum gamma-globulin concentrations more than twice normal and

sometimes antinuclear antibodies and/or anti-smooth muscle (anti-actin)

antibodies. Patients with another subtype may have normal or only slightly

elevated serum gamma-globulin concentrations but will have antibodies

against a particular cytochrome p450 isoenzyme that are called anti-LKM

(liver kidney microsome).

Patients in whom a diagnosis of autoimmune hepatitis is suspected should

have a liver biopsy. If the biopsy is consistent, treatment with steroids

(prednisone or pednisolone) and azathioprine (Imuran) is begun

immediately. These are tapered over the next 6 to 24 months depending upon

the patient's course. If immediate liver biopsy is contraindicated because

of a prolonged prothrombin time or thrombocytopenia, steroids and

azathioprine should be started prior to biopsy if the diagnosis of

autoimmune hepatitis is likely based on clinical criteria (e.g. a young

woman with severe hepatitis, elevated serum gamma-globulin concentration,

negative risk factors and serologies for viral hepatitis). The patient

will often rapidly improve and biopsy should be performed to confirm the

diagnosis as soon as the prothrombin time decreases and platelet count

increases to within safe ranges.

About two thirds to three quarters of patients with autoimmune hepatitis

respond to treatment based on the return of serum ALT and AST activities

to normal and an improved biopsy after several months. Some patients

relapse as steroids and azathioprine doses are tapered or stopped and need

chronic maintenance medications. Over the long term, many patients develop

cirrhosis despite having a response to treatment, and patients who do not

respond to treatment will almost always progress to cirrhosis. If

end-stage liver disease develops, orthotopic liver transplantation is an

effective procedure.

For more information, you may want to see the following review articles:

Czaja, A.J. and Freese, D. K. 2002. Diagnosis and treatment of autoimmune

hepatitis. Hepatology. 36:479-497.

Krawitt, E. L. 1996. Autoimmune hepatitis. New England Journal of

Medicine. 334:897-903.

Click here to return to Diseases of the Liver home page.

Click here to go to COLUMBIA UNIVERSITY GASTROENTEROLOGY WEB.

Copyright, 1995, 1996, 1998, 2002, J. Worman, M. D.

Autoimmune Hepatitis/ J. Worman, M. D./hjw14@...

" and the beat goes on....... " Sonny Bono " It's not the years in your life that

count. It's the life in your years. " Abraham Lincoln

__________________________________________________