Hepatitis is a general term indicating inflammation of the liver. Hepatitis can be caused by viruses, drugs, lack of blood flow (ischemia) and in some cases the immune system attacks the liver causing “autoimmune hepatitis.” Autoimmune hepatitis can occur in all age groups and both genders, however, it is most common in young adults and those in the fourth-sixth decades and is more common in women with an 3.6:1 female to male predominance (1-3).
Autoimmune hepatitis can be subcategorized into two primary subtypes. Type 1 autoimmune hepatitis is characterized by circulating antibodies to nuclei (ANA) and or smooth muscle (ASMA). Type 1 autoimmune hepatitis, also known as lupoid or classic autoimmune hepatitis, is the most common. Type 2 autoimmune hepatitis is defined by the presence of antibodies to liver/kidney microsomes and is seen primarily in girls and young women (4,5). Lastly, there are overlap conditions in which autoimmune hepatitis co-exists with autoimmune cholangiopathy and/or primary biliary cirrhosis (typically has antimitochondrial antibody). In autoimmune hepatitis the above mentioned circulating antibodies are targeted at liver cell (hepatocyte) antigens and there is typically elevated serum globulin concentrations. It is not known whether this is acquired or is a primary dysregulation of the immune system.
The most common symptom of autoimmune hepatitis is fatigue. Those affected, can present with jaundice (yellowing of the skin/eyes), dark urine or light colored stools, abdominal pain, joint pain, itching, and cirrhosis (scarring of the liver) (6). In some cases there are few or no symptoms suggestive of liver disease (7,8) while at the most severe end of the spectrum acute liver failure may occur manifested by confusion and a predisposition to bleeding (9-12).
Autoimmune hepatitis is typically diagnosed by using a combination of serologic/blood tests as above with a liver biopsy. The liver biopsy will allow for the evaluation of alternate diagnosis while at the same time providing a baseline assessment of the degree of damage the liver has sustained. Classic changes on liver biopsy that support the diagnosis of autoimmune hepatitis include “lymphoplasmacytic infiltrate” and “interface hepatitis”.
Treatment for autoimmune hepatitis is not indicated for all patients with this diagnosis. The American Association for the Study of Liver Disease has established a guideline to help your physician to determine if treatment is right for you. The most common treatments used in autoimmune hepatitis include glucocorticoids which reduce inflammation and will decrease scarring (fibrosis) over time. Glucocorticoids may result in weight gain, insomnia, elevated blood pressure, osteoporosis, insomnia and an increased risk of infection. In those with diabetes, blood sugars are often more difficult to control while taking glucocorticoids. To reduce the likelihood of these side effects, immune suppressing agents such as azathioprine or 6-mercaptopurine are often added to the treatment (13-15).
For more information on autoimmune hepatitis, please consult your Borland Groover gastroenterologist.
References
1. Czaja AJ, dos Santos RM, Porto A, Santrach PJ, Moore SB. Immune phenotype of chronic liver disease. Dig Dis Sci 1998;43:2149-2155.
2. Czaja AJ, Donaldson PT. Gender effects and synergisms with histocompatibility leukocyte antigens in type 1 autoimmune hepatitis. Am J Gastroenterol 2002;97:2051-2057.
3. Al-Chalabi T, Underhill JA, Portmann BC, McFarlane IG, Heneghan MA. Impact of gender on the long-term outcome and survival of patients with autoimmune hepatitis. J Hepatol 2008;48:140-147.
4. Manns M, Gerken G, Kyriatsoulis A, Staritz M, Meyer zum Buschenfelde KH. Characterization of a new subgroup of autoimmune chronic active hepatitis by autoantibodies against a soluble liver antigen. Lancet 1987;1:292-294.
5. Stechemesser E, Klein R, Berg PA. Characterization and clinical relevance of liver pancreas antibodies in autoimmune hepatitis. Hepatology 1993;18:1-9.
6. Czaja AJ. Diverse manifestations and evolving treatments of autoimmune hepatitis. Minerva Gastroenterol Dietol 2005;51:313-333.
7. Kogan J, Safadi R, Ashur Y, Shouval D, Ilan Y. Prognosis of symptomatic versus asymptomatic autoimmune hepatitis: a study of 68 patients. J Clin Gastroenterol 2002;35:75-81.
8. Feld JJ, Dinh H, Arenovich T, Marcus VA, Wanless IR, Heathcote EJ. Autoimmune hepatitis: effect of symptoms and cirrhosis on natural history and outcome. Hepatology 2005;42:53-62.
9. Kessler WR, Cummings OW, Eckert G, Chalasani N, Lumeng L, Kwo PY. Fulminant hepatic failure as the initial presentation of acute autoimmune hepatitis. Clin Gastroenterol Hepatol 2004;2:625-631.
10. Kessler, WR, Cummings, OW, Eckert, G, et al. Fulminant hepatic failure as the initial presentation of acute autoimmune hepatitis. Clin Gastroenterol Hepatol 2004; 2:625.
11. Bower, WA, Johns, M, Margolis, HS, et al. Population-based surveillance for acute liver failure. Am J Gastroenterol 2007; 102:2459.
12. Ichai P, Duclos-Vallee JC, Guettier C, Hamida SB, Antonini T, Delvart V, Saliba F, et al. Usefulness of Corticosteroids for the treatment of severe and fulminant forms of autoimmune hepatitis. Liver Transpl 2007;13:996-1003.
13. Cook GC, Mulligan R, Sherlock S. Controlled prospective trial of corticosteroid therapy in active chronic hepatitis. Q J Med 1971;40:159-185.
14. Soloway RD, Summerskill WH, Baggenstoss AH, Geall MG, Gitnick GL, Elveback IR,Schoenfield LJ. Clinical, biochemical, and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis. Gastroenterology 1972;63:820-833.
15. Murray-Lyon IM,Stern RB,Williams R.Controlled trial of prednisone and azathioprine inactive chronic hepatitis. Lancet 1973;I:735-737.