J Autoimmun. 2013 Mar;41:126-39. doi: 10.1016/j.jaut.2012.11.002. Epub 2012 Dec 4.

Autoimmune hepatitis: a comprehensive review.

Liberal R1, Grant CR, Mieli-Vergani G, Vergani D.
Autoimmune hepatitis (AIH) is an immune-mediated liver disorder characterised by female
preponderance, elevated transaminase and immunoglobulin G levels, seropositivity for autoantibodies
and interface hepatitis. Presentation is highly variable, therefore AIH should be considered during the
diagnostic workup of any increase in liver enzyme levels. Overlap/variant forms of the disease,
presenting with concomitant features of primary biliary cirrhosis or primary sclerosing cholangitis are
increasingly recognised. AIH is exquisitely responsive to immunosuppressive treatment, which should
be instituted promptly to prevent rapid deterioration and promote remission and long-term survival.
Difficult-to-treat or non-responsive patients should be treated with mycophenolate mofetil or, failing
that, calcineurin inhibitors. Persistent failure to respond or lack of adherence to treatment result in endstage liver disease. These patients, and those with fulminant liver failure (encephalopathy grade II-IV)
at diagnosis, will require liver transplantation. The pathogenesis of AIH is not fully understood,
although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired
immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack.
Liver damage is thought to be mediated primarily by CD4(pos) T-cells, although recent studies support
the involvement of diverse populations, including Th17 cells. Animal models faithfully representing the
human condition are needed to unravel the contribution of innate and adaptive, effector and regulatory
immune responses. A deeper understanding of the pathogenesis of AIH is likely to contribute to the
development of novel treatments, such as the adoptive transfer of autologous expanded antigenspecific regulatory T-cells, which ultimately aim to restore tolerance to liver-derived antigens.

Rev Col Gastroenterol vol.27 no.4 Bogot Oct./Dec. 2012

Autoimmune hepatitis is a condition which can be asymptomatic or can present as acute hepatitis or
liver cirrhosis. Diagnosis is based on clinical criteria and laboratory criteria. Laboratory criteria include
elevated levels of immunoglobulin G and/or autoantibodies and histological criteria such as hepatitis
interface, the presence of plasma cells and lymphocytic infiltrate. In difficult to diagnose cases original
or modified scoring systems can be used. Treatment is based on the use of immunosuppressants
such as corticosteroids and azathioprine that have changed the natural history of disease.

A.D.A.M. Medical Encyclopedia.

Autoimmune hepatitis
Lupoid hepatitis; Chronic acute liver disease
Last reviewed: October 8, 2012.

Autoimmune hepatitis is inflammation of the liver that occurs when immune cells mistake the liver's
normal cells for harmful invaders and attack them.
Causes, incidence, and risk factors
This form of hepatitis is an autoimmune disease. The body's immune system can't tell the difference
between healthy body tissue and harmful, outside substances. The result is an immune response that
destroys normal body tissues.
Liver inflammation, or hepatitis may occur along with other autoimmune diseases, including:
Graves disease
Inflammatory bowel disease
Rheumatoid arthritis
Scleroderma
Sjogren syndrome
Systemic lupus erythematosus
Thyroiditis
Type 1 diabetes
Ulcerative colitis
Autoimmune hepatitis sometimes occurs in relatives of people with autoimmune diseases. This
suggests that there may be a genetic cause. This disease is most common in young girls and women.

Revista de Gastroenterologa del Per

versin impresa ISSN 1022-5129
Rev. gastroenterol. Per v.24 n.4 Lima oct./dic. 2004
Chronic liver disease is a clinical entity of different origins. It is most frequently caused by viral
infection and alcohol consumption. The entities of immunological origin are listed in third place
including autoimmune hepatitis, primary biliar cirrhosis, primary sclerosing cholangitis, as well as
superposition syndromes. In Peru report of cases relating to autoimmune hepatitis are very few and its
frequency is unknown. In 2002, autoimmune etiology represented 13% of all the cases admitted in the
Hepathology Unit of Edgardo Rebagliati Martins National Hospital (HNERM) for chronic hepatic
disease.
In this article, 30 cases of autoimmune hepatitis clinically and serologically diagnosed are reported.
Biopsy was performed on 97% of the cases, of which 70% showed cirrhosis. The relationship F/M was
5/1, the average age was 48.59 years, and in 6.7% of the cases the initial picture was acute hepatic
insufficiency. Antinuclear antibodies were found in 73.33%, smooth antimuscle antibodies in 43.33%,
and antimitochondrial antibodies in 16.7%, with a coexistence of autoantibodies in 40%. The

endoscopy performed revealed the presence of varices in 20% of the cases, but only one case of
variceal hemorrhage. In most cases, therapy was initiated based on prednisone and azathioprine. Of
26 cases that were treated, 80% had an initial remission, 2 responded partially, and 3 did not respond.
There were complications related to the treatment with immunosuppressants in 16.7% of the cases,
and especially severe infections in 3 cases. In conclusion, autoimmune hepatitis is a substantial cause
of chronic hepatic disease that has similar clinical characteristics to those reported in international
medical journals. In most cases it responds to treatment with immunosuppressants. However,
adequate follow-up is recommended to detect secondary complications in the treatment with
immunosuppressants, especially in infections which represent a high risk of mortality in the
immunosuppressed patient.

Revista Cubana de Medicina

versin On-line ISSN 1561-302X
Rev cubana med v.39 n.1 Ciudad de la Habana ene.-mar. 2000
This necroinflammatory liver disease of unknown origin was reported for the first time in 1950. It was
mainly described in young women. This nosological entity has had several denominations through
history. At present, it is known as autoimmune hepatitis. It appears in individuals with certain genetic
predisposition when they are exposed to some environmental agent that has not been identified yet,
giving rise to an autoimmune mechanism directed to the liver, where a necroinflammatoy process
takes place leading to fibrosis and, finally, to cirrhosis. There are 3 types of autoimmune hepatitis:
type1 is characterized by the presence of antinuclear antibodies, smooth antimuscle and antiactin;
type 2, by the existance of anti- liver/kidney chromosome antibodies; and type 3, by the appearance of
antibodies against the soluble antigen. The diagnosis is based on the histological study of the liver and
on the presence of the above mentioned antibodies. Monoclonal hypergammaglobulinemia is a
laboratory alteration that characterizes the disease. Other lab findings were the elevation of
aminotranferases, alkaline phosphatase and bilirubin. The response to the treatment with
glucocorticoids is common in this affection. Azathioprine associated with corticosteroids is used in the
maintenance therapy in order to prescribe low doses and to avoid their side effects. Azathioprine may
be also used alone as a maintenance therapy.