Autoimmune hepatitis: Difference between revisions
(Created page with "Autoimmune hepatitis (AIH) is a cause of hepatitis which presents acutely but becomes chronic, possibly progressing to cirrhosis. AIH mostly affects females. It can present at any year but mostly begins in the teens or in elderly. As with most autoimmune diseases, there is associated with other autoimmune diseases. It has a good response to therapy and a good prognosis, but a 40% mortality rate if untreated. 70% of patients relapse at some point after having reached re...") |
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Autoimmune hepatitis (AIH) is a cause of hepatitis which presents acutely but becomes chronic, possibly progressing to cirrhosis. | '''Autoimmune hepatitis''' (AIH) is a cause of [[hepatitis]] which presents acutely but becomes chronic, possibly progressing to [[cirrhosis]]. | ||
AIH mostly affects females. It can present at any | AIH mostly affects females. It can present at any age but mostly begins in the teens or in elderly. As with most autoimmune diseases, there is associated with other autoimmune diseases. | ||
It has a good response to therapy and a good prognosis, but a 40% mortality rate if untreated. 70% of patients relapse at some point after having reached remission. | It has a good response to therapy and a good prognosis, but a 40% mortality rate if untreated. 70% of patients relapse at some point after having reached remission. | ||
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== Clinical features == | == Clinical features == | ||
AIH may present asymptomatically (only abnormal laboratory parameters), as acute hepatitis, or with cirrhosis. | AIH may present asymptomatically (only abnormal laboratory parameters), as acute [[hepatitis]], or with [[cirrhosis]]. | ||
Some extrahepatic manifestations of AIH include: | Some extrahepatic manifestations of AIH include: | ||
* Vitiligo | * [[Vitiligo]] | ||
* Acne | * [[Acne]] | ||
* Vasculitis | * [[Vasculitis]] | ||
* Erythema nodosum | * [[Erythema nodosum]] | ||
* Rheumatological disease | * Rheumatological disease | ||
== Diagnosis and evaluation == | == Diagnosis and evaluation == | ||
As with any hepatitis, liver enzymes (AST, ALT, GGT, ALP) are elevated. AST and ALT may be significantly elevated, often >10x the upper limit of normal. However, the following findings are typical for AIH: | As with any hepatitis, [[Liver enzyme|liver enzymes]] (AST, ALT, GGT, ALP) are elevated. AST and ALT may be significantly elevated, often >10x the upper limit of normal. However, the following findings are typical for AIH: | ||
* Autoantibodies | * Autoantibodies | ||
** Anti-nuclear antibody (ANA) | ** [[Anti-nuclear antibody]] (ANA) | ||
** Anti-smooth muscle antibody (SMA) | ** [[Anti-smooth muscle antibody]] (SMA) | ||
* Hypergammaglobulinaemia | * [[Hypergammaglobulinaemia]] | ||
Biopsy and histology are obligatory to confirm the diagnosis, to determine the activity and stage, and to exclude other diseases. Typical for AIH is lymphocytic infiltration in the portal tract. | Biopsy and histology are obligatory to confirm the diagnosis, to determine the activity and stage, and to exclude other diseases. Typical for AIH is lymphocytic infiltration in the portal tract. | ||
== Treatment == | == Treatment == | ||
Immunosuppressive therapy to induce and maintain remission is indicated, in all but very mild cases. Remission is induced with | [[Immunosuppressive therapy]] to induce and maintain remission is indicated, in all but very mild cases. Remission is induced with [[corticosteroids]] ± [[azathioprine]] and maintained with azathioprine or other immunosuppressants. Once remission has been maintained for a long time (>18 months), drug withdrawal may be attempted as many don’t require long-term immunosuppressive therapy to prevent relapse. | ||
In very severe cases, liver transplantation is an option. | In very severe cases, [[liver transplantation]] is an option. | ||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
Latest revision as of 15:28, 27 October 2023
Autoimmune hepatitis (AIH) is a cause of hepatitis which presents acutely but becomes chronic, possibly progressing to cirrhosis.
AIH mostly affects females. It can present at any age but mostly begins in the teens or in elderly. As with most autoimmune diseases, there is associated with other autoimmune diseases.
It has a good response to therapy and a good prognosis, but a 40% mortality rate if untreated. 70% of patients relapse at some point after having reached remission.
Etiology
The development of AIH often involves an environmental trigger (viral infection, drug, etc.) in a genetically susceptible person. The following alleles are associated with AIH:
- HLA-B8
- HLA-DR3
- HLA-DR4
Clinical features
AIH may present asymptomatically (only abnormal laboratory parameters), as acute hepatitis, or with cirrhosis.
Some extrahepatic manifestations of AIH include:
- Vitiligo
- Acne
- Vasculitis
- Erythema nodosum
- Rheumatological disease
Diagnosis and evaluation
As with any hepatitis, liver enzymes (AST, ALT, GGT, ALP) are elevated. AST and ALT may be significantly elevated, often >10x the upper limit of normal. However, the following findings are typical for AIH:
- Autoantibodies
- Anti-nuclear antibody (ANA)
- Anti-smooth muscle antibody (SMA)
- Hypergammaglobulinaemia
Biopsy and histology are obligatory to confirm the diagnosis, to determine the activity and stage, and to exclude other diseases. Typical for AIH is lymphocytic infiltration in the portal tract.
Treatment
Immunosuppressive therapy to induce and maintain remission is indicated, in all but very mild cases. Remission is induced with corticosteroids ± azathioprine and maintained with azathioprine or other immunosuppressants. Once remission has been maintained for a long time (>18 months), drug withdrawal may be attempted as many don’t require long-term immunosuppressive therapy to prevent relapse.
In very severe cases, liver transplantation is an option.