Autoimmune hepatitis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Medical Therapy

Mainstay treatment of autoimmune hepatitis is pharmacotherapy. Corticosteroids alone or in combination with immunosuppressants are commonly used.

Acute Pharmacotherapies

According to American Association for the Study of Liver Diseases indications for immunosuppressive treatment:

Indications for Immunosuppressive Treatment
Absolute Indications Relative Indications None
Serum AST >10 fold upper limit of normal range(ULN) Symptoms like fatigue, arthralgia, jaundice Asymptomatic with normal or near normal serum

AST and gamma globulin levels

Serum AST >5 fold ULN Serum AST and/or gamma globulin less than absolute criteria Inactive cirrhosis or mild portal inflammation

(portal hepatitis)

Gamma globulin level>2 fold ULN Interface hepatitis Severe cytopenia (white blood cell counts

<2.5 x109/L or platelet counts <50 x 109/L)

Bridging necrosis or multiacinar

necrosis on histological examination

Osteopenia, emotional instability, hypertension, diabetes,

or cytopenia (white blood cell counts <2.5 x109/L

or platelet counts <50 x109/L)

complete deficiency of TPMT activity

precludes treatment with azathioprine

Incapacitating symptoms such as fatigue

and arthralgia

Vertebral compression, psychosis, brittle diabetes,

uncontrolled hypertension, known intolerances

to prednisone or azathioprine

According to American Association for the Study of Liver Diseases, Immunosuppressive Treatment Regimens for Adults in Autoimmune Hepatitis:
Immunosuppressive Treatment Regimens for Adults in Autoimmune Hepatitis
Monotherapy

Prednisone only* (mg/day)

Weeks Dosage
First 60
Second 40
Third 30
Fourth 30
Maintenance until endpoint 20 and below
  • Despite our clear understanding of the pathogenesis and pathophysiology of AH, approximately 80 % of patients will have a good initial response to corticosteroids, with a ten-year survival rate approaching 90%.
  • The decision to treat patients with only mild disease is often based on symptoms.
  • Asymptomatic patients with mild inflammation on Bx can be observed with careful monitoring of histology.
  • The goal of therapy is generally normalization of both transaminases and histology, and in general, most patients who are going to respond do so by 6 months.
  • Unfortunately, the results with alternate-day or pulsed steroid regimens have been disappointing and daily regimens are preferred.
  • Azathioprine can be used as a steroid-sparing agent.
  • Additionally, some authors recommend using lower prednisone doses with the goal of partial suppression of disease, as opposed to higher doses of steroids with the goal being remission.
  • For the 10 – 20% of patients who develop progressive disease despite steroids and/or azathioprine, cyclosporine and tacrolimus have recently been shown to induce remission.
  • There are no firm guidelines concerning the tapering or discontinuation of therapy.

Contraindicated medications

Autoimmune hepatitis is considered an absolute contraindication to the use of the following medications:

Recommendations for the Treatment of Autoimmune Hepatitis (DO NOT EDIT)

  1. Immunosuppressive treatment based on serum aspartate aminotransferase (AST), serum alanine aminotransferase (ALT), serum gamma-globulin levels, and histological features
    1. Prednisone or prednisolone with azathioprine (adults)
    2. Prednisone with azathioprine or 6-mercaptopurine (children)
    3. Prednisone or prednisolone alone
  2. Monitoring for bone disease
  3. Adjunctive therapies for bone disease (weight bearing exercise program, vitamin D and calcium supplementation, bisphosphonates)
  4. Pretreatment vaccination against hepatitis A virus (HAV) and hepatitis B virus (HBV)
  5. Management of treatment side effects and risks, including during pregnancy
  6. Alternative drug therapies for suboptimal response (cyclosporine, tacrolimus, or mycophenolate mofetil)
  7. Hepatic ultrasonography to detect hepatocellular carcinoma (HCC)
  8. Liver transplantation, management of recurrent disease after transplant with drug therapy and/or retransplantation in certain patients

References

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