Oettinger et al. published a series of 142 children with autoimmune hepatitis [8]. Findings included:

• Jaundice 58%
• Non specific weakness 57%
• Anorexia 47%
• Abdominal pain 38% 
• Paleness 26%

Other symptoms and signs may include:

• General discomfort or uneasiness 
• Abdominal distension
• Pruritis
• Diarrhea
• Edema
• Weight loss
• Spider angiomata
• Hepatomegaly
• Splenomegaly
• Portal hypertension (advanced cases)
• CNS involvement (advanced cases)

• Pain

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice Enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools Treatment If diagnosed early, medication [cincinnatichildrens.org]

  We report a case with torpid evolution, presented with multiple and deep ulcers in a young patient with autoimmune hepatitis, causing pain and significant disability. We observed complete healing of lesions after two months of successful treatment. [ncbi.nlm.nih.gov]

  Pain in stomach Stomach pain [ more ] 0002027 Arthralgia Joint pain 0002829 Chronic fatigue Chronic extreme exhaustion 0012432 Depressivity Depression 0000716 Spider hemangioma 0012522 5%-29% of people have these symptoms Acute hepatitis Acute liver [rarediseases.info.nih.gov]

  Symptoms of autoimmune hepatitis Symptoms are similar to other forms of hepatitis. abdomen pain enlarged liver fatigue itchy skin jaundice joint pain light colored stools (often described as "clay-colored") nausea and vomiting urine that is darker than [vod.alternativli.co.il]

  Stomach pain Bloated belly Bruises and bleeding Vomiting Dark-colored urine Light-colored stools Loss of menstrual periods in women Get useful, helpful and relevant health + wellness information enews More health news + info [my.clevelandclinic.org]

• Fatigue

  Dextroamphetamine sulfate was used to treat edema, myalgia, and chronic fatigue associated with autoimmune hepatitis (AIH). Sympathomimetic amine therapy completely abrogated the symptoms associated with AIH. [ncbi.nlm.nih.gov]

  The patient suffers from non-specific symptoms such as fatigue, dark urine, and discoloration of stools, upper abdominal discomfort or skin rashes. [drfalkpharma.com]

  Common initial symptoms include fatigue or muscle aches or signs of acute liver inflammation including fever, jaundice, and right upper quadrant abdominal pain. [en.wikipedia.org]

• Ascites

  Her history was significant for por- tal hypertension, encepha- lopathy, ascites, coagulopathy, hyperammonemia, and end-stage liver disease secondary to AIH. [ncbi.nlm.nih.gov]

  We provide pre- and post-liver transplantation evaluations and care, and treat patients with complications of portal hypertension, such as variceal bleeding, ascites and encephalopathy, with an array of endoscopic and diagnostic procedures. [northwell.edu]

  Fluid in your abdomen (ascites). Liver disease can cause large amounts of fluid to accumulate in your abdomen. Ascites can be uncomfortable and may interfere with breathing and is usually a sign of advanced cirrhosis. Liver failure. [mayoclinic.org]

• Fever

  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. [texasliver.com]

  Strontium ranelate, which is used for postmenopausal osteoporosis, has been associated with drug rash with eosinophilia and systemic symptoms (DRESS) syndrome, a severe, acute, potentially fatal, multisystem adverse drug reaction characterized by skin rash, fever [ncbi.nlm.nih.gov]

  Symptoms include jaundice, dark urine, fever and flulike symptoms. It can be prevented with hepatitis A vaccine. A virus that causes liver disease and inflammation. [bshr.com]

• Weight Loss

  A 25-year-old woman presented with jaundice, palpitation, and weight loss of 5 kg during a period of 2 weeks. [ncbi.nlm.nih.gov]

  Cell Transplant Liver Cancer Liver & Biliary Diseases Pancreatic Diseases Pancreatic Cancer Pancreatitis Peptic Ulcer Disease Portal Hypertension Primary Biliary Cirrhosis (PBC) Primary Sclerosing Cholangitis Stomach (Gastric) Cancer Stomach Diseases Weight [nyp.org]

  Findings included: Jaundice 58% Non specific weakness 57% Anorexia 47% Abdominal pain 38% Paleness 26% Other symptoms and signs may include: General discomfort or uneasiness Abdominal distension Pruritis Diarrhea Edema Weight loss Spider angiomata Hepatomegaly [symptoma.com]

  Symptoms can develop over weeks or months and are non-specific (tiredness, general fatigue, anorexia, weight loss and joint and muscle pains). On rare occasions AIH develops quickly and symptoms and signs appear rapidly. [royalfree.nhs.uk]

• Mild Clinical Course

  The ulcerative colitis typically involves a majority of the colon, but often has a relatively mild clinical course. [doi.org]

• Nausea

  We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. [ncbi.nlm.nih.gov]

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice Enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools Treatment If diagnosed early, medication [cincinnatichildrens.org]

  Share on Pinterest Symptoms of autoimmune hepatitis can include nausea, abdominal pain, and fatigue. Symptoms of autoimmune hepatitis can vary. [medicalnewstoday.com]

  Symptoms of autoimmune hepatitis Symptoms are similar to other forms of hepatitis. abdomen pain enlarged liver fatigue itchy skin jaundice joint pain light colored stools (often described as "clay-colored") nausea and vomiting urine that is darker than [vod.alternativli.co.il]

  Some patients do not have any symptoms, while others may have such symptoms as: Tiredness/low energy Nausea Itchy skin or rash Yellow-colored skin or whites of the eyes (jaundice) As scarring in the liver continues and liver function gets worse, the patient [my.clevelandclinic.org]

• Vomiting

  We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. [ncbi.nlm.nih.gov]

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice Enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools Treatment If diagnosed early, medication [cincinnatichildrens.org]

  Symptoms of autoimmune hepatitis Symptoms are similar to other forms of hepatitis. abdomen pain enlarged liver fatigue itchy skin jaundice joint pain light colored stools (often described as "clay-colored") nausea and vomiting urine that is darker than [vod.alternativli.co.il]

  Tiredness/low energy Nausea Itchy skin or rash Yellow-colored skin or whites of the eyes (jaundice) As scarring in the liver continues and liver function gets worse, the patient may notice: Joint pain Stomach pain Bloated belly Bruises and bleeding Vomiting [my.clevelandclinic.org]

• Abdominal Pain

  Common presenting symptoms included jaundice, fatigue, and abdominal pain. The majority of subjects received steroids for induction and AZA for maintenance of remission. [ncbi.nlm.nih.gov]

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice Enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools Treatment If diagnosed early, medication [cincinnatichildrens.org]

  Share on Pinterest Symptoms of autoimmune hepatitis can include nausea, abdominal pain, and fatigue. Symptoms of autoimmune hepatitis can vary. [medicalnewstoday.com]

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice An enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools In women, loss of menstruation [columbiasurgery.org]

• Right Upper Quadrant Pain

  Common initial symptoms include fatigue or muscle aches or signs of acute liver inflammation including fever, jaundice, and right upper quadrant abdominal pain. [en.wikipedia.org]

  Acute AIH was defined as the recent onset of hepatitis symptoms (jaundice, fatigue, right upper quadrant pain, and/or anorexia) for less than six months and a minimal 10-fold rise in serum transaminases relative to the upper limit of normal values in [elsevier.es]

  One-third of patients present with an acute hepatitis with jaundice and right upper quadrant pain or occasionally fulminant hepatic failure. 6 The remainder are diagnosed either as the result of incidental findings on routine liver biochemistry tests [bmb.oxfordjournals.org]

  One-third of patients present with an acute hepatitis with jaundice and right upper quadrant pain or occasionally fulminant hepatic failure.6 The remainder are diagnosed either as the result of incidental findings on routine liver biochemistry tests in [academic.oup.com]

  Almost half are asymptomatic at diagnosis; 40 symptomatic patients may present with jaundice, pruritis, and right upper quadrant pain. [doi.org]

• Jaundice

  A possibility of DIAIH should be considered in cases of DILI which exhibit overt jaundice, autoantibodies, intense histological inflammation and a long latency period. [ncbi.nlm.nih.gov]

  A 43-year-old Caucasian male patient with a past medical history of hypertension was admitted due to abdominal pain of two-week duration and progressive jaundice. [bloodjournal.org]

  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. [texasliver.com]

• Hepatomegaly

  CT Nonspecific, ranging from normal to hepatomegaly and cirrhosis. [radiopaedia.org]

  Findings included: Jaundice 58% Non specific weakness 57% Anorexia 47% Abdominal pain 38% Paleness 26% Other symptoms and signs may include: General discomfort or uneasiness Abdominal distension Pruritis Diarrhea Edema Weight loss Spider angiomata Hepatomegaly [symptoma.com]

  AIH symptoms include: enlarged liver (hepatomegaly) abnormal blood vessels on the skin (spider angiomas) abdominal distention (swelling) dark urine pale-colored stools Additional symptoms may include: yellowing of the skin and eyes (jaundice) itching [healthline.com]

• Recurrent Jaundice

  Other symptoms may include: Fever Abdominal pain Arthritis Anemia Dry eyes and mouth Recurrent jaundice frequently develops in severe cases Lack of menstrual periods Normally, these symptoms of autoimmune disease develop gradually over the course of weeks [premiermedicalhv.com]

• Pruritus

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice Enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools Treatment If diagnosed early, medication [cincinnatichildrens.org]

  Weight loss and intense pruritus (unusual). Signs Common findings on physical examination are as follows: Hepatomegaly. Jaundice (around 50% of patients). Splenomegaly. Spider angiomata. Ascites. Encephalopathy. [patient.info]

  Fatigue Abdominal pain Joint pain Itching (pruritus) Jaundice An enlarged liver Abnormal blood vessels on the skin (spider angiomas) Nausea and vomiting Loss of appetite Skin rashes Dark-colored urine, light colored stools In women, loss of menstruation [columbiasurgery.org]

  Presentation Symptoms range from minor to severe can be acute or chronic fatigue abdominal pain joint pain pruritus skin rashes dark-colored urine amenorrhea in women - often an initial complaint Physical exam inspection distended abdomen - ascites jaundice [step2.medbullets.com]

• Hirsutism

  • Endocrine changes like cushingoid appearance, acne, hirsutism. • Pulmonary changes like pleurisy,transitory pulmonary infiltrates and collapse. • Acute,recurrent and non- deforming polyarthrits of large joints. • Mild aneamia, leucopenia, thrombocytopenia [de.slideshare.net]

  Skin rashes (including acne), hirsutism. Oedema. Amenorrhoea. Chest pain (pleuritis). Weight loss and intense pruritus (unusual). Signs Common findings on physical examination are as follows: Hepatomegaly. Jaundice (around 50% of patients). [patient.info]

  Compared to prednisone, it has a relatively safe adverse-effect profile with headache and respiratory infection seen most commonly. 9 Cosmetic changes such as moon face, acne, and hirsutism have been noted. 8 Budesonide is primarily metabolized through [uspharmacist.com]

  Common symptoms include the following: Fatigue Upper abdominal discomfort Mild pruritus Anorexia Myalgia Diarrhea Cushingoid features Arthralgias Skin rashes (including acne) Edema Hirsutism Amenorrhea Chest pain from pleuritis Weight loss and intense [emedicine.medscape.com]

• Yellow Discoloration of the Skin

  discoloration of the skin and the whites of the eyes ( jaundice ). [labtestsonline.org]

• Arthritis

  This report confirms the need to monitor liver enzymes carefully in patients using long-term treatment with MTX for psoriasis or rheumatoid arthritis. [ncbi.nlm.nih.gov]

  It usually presents in children with nonspecific symptoms, such as malaise, lethargy, and arthralgia without arthritis. [pedinfect.com]

  About half the people with type 1 autoimmune hepatitis have other autoimmune disorders, such as celiac disease, rheumatoid arthritis or ulcerative colitis. Type 2 autoimmune hepatitis. [mayoclinic.org]

• Arthralgia

  At the time of diagnosis of AIH, patients presented with arthralgia, abdominal complaints, cutaneous involvement and fatigue as common symptoms. All patients fulfilled the current diagnostic criteria of both, AIH and SLE. [ncbi.nlm.nih.gov]

  The patient will sometimes present with jaundice, fever and right upperquadrant pain and occasionally systemic symptoms such as arthralgias (arthritis), myalgias (muscle aches), polyserositits and thrombocytopenia. [texasliver.com]

  Although patients may present with acute symptoms (eg, arthralgia), about 34% to 45% are asymptomatic and are identified subsequent to abnormal liver function tests such as increased alanine and/or aspartate aminotransferase (generally 2,3 Elevated levels [questdiagnostics.com]

  The next most common symptoms are abdominal pain, jaundice, arthralgia, and epistaxis. [clinicaladvisor.com]

• Lethargy

  […] lifelong immunosuppression Genetics AIH-1 – DRB1 *0301, DRB1 *0401 AIH-2 – DQB1 *0201, DRB1 *07, DRB1 *03 Clinical Presentation ~25% of patients are asymptomatic when AIH is detected by liver function testing (Liberal, 2014) Nonspecific symptoms – fatigue, lethargy [arupconsult.com]

  […] asymptomatic (12–35% of the cases), with signs of chronic liver disease, or acute or even fulminant hepatic failure.[5][6] People usually present with one or more nonspecific symptoms, sometimes of long lasting duration, as fatigue, general ill health, lethargy [en.wikipedia.org]

  It usually presents in children with nonspecific symptoms, such as malaise, lethargy, and arthralgia without arthritis. [pedinfect.com]

  […] is rare and appears to be more common in AIH-2. 7 The common clinical presentation of AIH is characterized by one or more of the following nonspecific symptoms of varying severity: fatigue, general ill health, mild pain in the right upper quadrant, lethargy [uspharmacist.com]

• Dark Urine

  The patient suffers from non-specific symptoms such as fatigue, dark urine, and discoloration of stools, upper abdominal discomfort or skin rashes. [drfalkpharma.com]

  Symptoms include: Fatigue Weakness Itching Nausea Vomiting Fever Diarrhea Abdominal pain on the right side of the body where the liver is located Jaundice (yellow skin and eyes) Dark urine Diagnosing Autoimmune Hepatitis Your hepatologist will first perform [virginiamason.org]

  Symptoms Symptoms may include: Fatigue General discomfort, uneasiness, or ill feeling (malaise) Itching Loss of appetite Nausea or vomiting Joint pain Pale or clay-colored stools Dark urine Abdominal distention Absence of menstruation (amenorrhea) may [ufhealth.org]

  AIH symptoms include: enlarged liver (hepatomegaly) abnormal blood vessels on the skin (spider angiomas) abdominal distention (swelling) dark urine pale-colored stools Additional symptoms may include: yellowing of the skin and eyes (jaundice) itching [healthline.com]

• Amenorrhea

  Symptoms Symptoms may include: Fatigue General discomfort, uneasiness, or ill feeling (malaise) Itching Loss of appetite Nausea or vomiting Joint pain Pale or clay-colored stools Dark urine Abdominal distention Absence of menstruation (amenorrhea) may [ufhealth.org]

  Presentation Symptoms range from minor to severe can be acute or chronic fatigue abdominal pain joint pain pruritus skin rashes dark-colored urine amenorrhea in women - often an initial complaint Physical exam inspection distended abdomen - ascites jaundice [step2.medbullets.com]

  Genetic predisposition: North America / Europe: HLA-DR3 (DRB1*0301) and HLA-DR4 (DRB1*0401) China / Japan: HLA-DR4 (DRB1*0405) Clinical features Most patients have nonspecific symptoms: fatigue, anorexia, nausea, weight loss, jaundice, pruritus and amenorrhea [pathologyoutlines.com]

  Absence of menstrual periods in women (amenorrhea). [rarediseases.info.nih.gov]

Work up consists of a detailed history and physical examination.

Laboratory Tests

• Complete blood count
• Liver function tests
• Serology for ANAs, SMAs, LKM1, anti-LC1
• Immunoglobulins and albumen levels
• Blood profile

Imaging Studies

• Liver biopsy
• CT scan

Test Results

Test results will indicate eosinophilia, raised ANA, ASMA and other increased antibody titres. Blood profile will show increased blood count and a prolonged prothrombin time. Biopsy will show Ag-Ab aggregates in the liver. CT scan will show extrahepatic involvement, if any.

• Hypoalbuminemia

  In a multivariate analysis, we observed that age at diagnosis up to 22 years [odds ratio (OR): 23.22; confidence interval (CI): 3.978-135.549; P CONCLUSION: Our study confirmed that younger age, hypoalbuminemia, increased MPV, and cirrhosis at diagnosis [ncbi.nlm.nih.gov]

  Evidence of synthetic dysfunction by an increased international normalized ratio and hypoalbuminemia was seen in ∼40% to 45% of cases, which correlates with histologic findings of cirrhosis in the study group. [pediatrics.aappublications.org]

  […] or the onset of hepatocellular carcinoma as a complication of cirrhosis Seropositive results for antinuclear antibodies (ANAs), smooth-muscle antibodies (SMAs), or liver-kidney microsomal type 1 (LKM-1) or anti–liver cytosol 1 (anti-LC1) antibodies Hypoalbuminemia [emedicine.medscape.com]

• Hyperglobulinemia

  Although patients may present with or without evidence of circulating autoantibodies, hyperglobulinemia is a rather consistent laboratory feature. [ncbi.nlm.nih.gov]

  PATHOGENESIS EVIDENCE SUPPORTING AUTOIMMUNE PATHOGENESIS  Histopathological lesions composed of cytotoxic Tcells and plama cells  Circulating autoantibodies  Hyperglobulinemia  Other autoimmune disorders: thyroiditis, RA, autoimmune hemolytic anemia [de.slideshare.net]

  Type 1 AIH, the most common type worldwide, is the classic syndrome occurring in young women, associated with marked hyperglobulinemia, lupoid features, circulating anti-nuclear antibodies (ANA) and/or anti-smooth muscle antibodies (ASMA), Human Leukocyte [scitechnol.com]

• Antinuclear Autoantibodies

  Autoantibodies Data regarding autoantibodies at diagnosis, including antinuclear antibody, smooth muscle antibody, liver/kidney microsomal, and LC1 antibodies, are provided in Table 2. [pediatrics.aappublications.org]

• Liver Enzymes Abnormal

  Liver Enzyme Abnormalities Elevation of liver transaminases less than 500 UI/L with normal alkaline phosphatase is typical. [clevelandclinicmeded.com]

• Liver Biopsy

  A 61-year-old male was referred to our hospital because of abnormal liver enzyme levels after initial diagnosis of CAEBV had been made by laboratory tests and liver biopsy. [ncbi.nlm.nih.gov]

  […] pretreatment liver biopsy. [tropicalgastro.com]

• HLA-DR3

  For example, HLA DR3 positivity only contributes when other antibodies are negative. This calculator correctly scores such circumstances. Also, it is not possible to check off boxes in an illogical fashion. [web.archive.org]

  Transgenic AIH mice carrying HLA-DR3 showed reduced diversity and total load of gut microbiota. [ncbi.nlm.nih.gov]

  […] association HLA DR3/DR4 HLA DR3/DR7 Prognosis Good More aggressive and classically harder to treat; rates of relapse are high Type 1 Type 2 Percentage of all AIH cases 80% 20% Geography Global More common in Northern Europe Gender (female : male) 4 : [bmb.oxfordjournals.org]

• HLA-DR4

  (DRB1*0301) and HLA-DR4 (DRB1*0401) China / Japan: HLA-DR4 (DRB1*0405) Clinical features Most patients have nonspecific symptoms: fatigue, anorexia, nausea, weight loss, jaundice, pruritus and amenorrhea Up to 25% of patients are asymptomatic, whereas [pathologyoutlines.com]

  Significance of HLA DR4 in type 1 autoimmune hepatitis. ] the differing findings regarding HLA association are noteworthy. [journal-of-hepatology.eu]

  HLA-DR3 serotype also occurs more commonly in caucasians. 10,11 HLA-DR4 serotype associated disease occurs more commonly in older adults and usually responds well to immunosuppressive therapy and exhibits more extra-hepatic manifestations. 10,11 HLA-DR4 [jpma.org.pk]

  Race-, sex-, and age-related differences in incidence The disease is most common in whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers. The Japanese population has a low frequency of HLA-DR3 markers. [emedicine.medscape.com]

• HLA-B8

  […] consumption Male Male 35-35 gm/day; female 25-40 gm/day (0 points) Male 50-80 gm/day; female 40-60 gm/day (-2 points) Male >80 gm/day; female >60 gm/day (-1 point) Genetic factors Other autoimmune diseases in patient or first-degree relatives (1 point) HLA [merckmanuals.com]

  On the other hand, patients with an HLA-B8 allele tend to have a more aggressive disease course. In the Italian population HLA-B8-DR3-DQ2, in Japan HLA-DRB1*0405 are the most frequent AIH associated haplotypes (12). [path.upmc.edu]

  Individuals with a “hepatitic” form of PBC frequently are positive for HLA B8, DR3, or DR4,66 and these genetic markers can be shared in all forms of autoimmune liver disease with a variable impact on disease behaviour.71-73 A diagnostic algorithm that [gut.bmj.com]

  In patients of European descent, AIH is associated with the HLA A1-B8-DR3 haplotype and particularly with DR3. [doi.org]

Medications

Corticosteroids are the mainstay of treatment. Budesonide has been shown to be more effective in inducing remission than prednisone, and results in fewer adverse effects [9].

Transplantation

Liver transplantation may be required if patients do not respond to drug therapy or when patients present with fulminant liver failure.

Autoimmune hepatitis may advance to cirrhosis [3]. Without treatment, almost 50% of patients will die within five years. Mild liver disease generally has a good prognosis with treatment, however, the more severe the disease, the worst the prognosis. In severe and/or complicated disease, life expectancy is greatly reduced.

Complications

Untreated and uncontrolled autoimmune hepatitis may progress to chronic hepatitis that will then show widely systemic manifestations. Complications include the following:

• Cirrhosis
• Esophageal varices
• Portal hypertension
• Coagulopathies
• Blood dyscrasias
• Pernicious anemia
• Ascites
• Encephalopathy
• Malnutrition
• Hepatosplenomegaly

Autoimmune hepatitis is an inflammation of the liver that occurs when immune cells mistake the liver's normal cells for harmful invaders and attack them [3]. The exact cause of the disease is unknown, but a number of factors have been implicated in its development. It may be initiated or triggered by genetic factors, viral infections (eg acute hepatitis A and B, Epstein-Barr virus infection) [4], etc. Some chemical factors such as interferons, etc may also play a role.

Genetic factors are proposed to be the main culprit behind this disease. DR3, DR4, B8, B15 and C4A gene deletions and mutations may lead to the production of autoantibodies. To some point, the body will be able to combat these autoantibodies but when an environmental trigger, such as infection by a virus, etc may cause the threshold of tolerance to be breached, then the disease becomes symptomatic.

Incidence

0.1-1.2 cases per 100,000 people are estimated to suffer from autoimmune hepatitis in the United States. 6% of all liver transplantations conducted in the US are due to this disease.

Age

It may occur at any age, ranging from infants to old people. Some studies reveal a bimodal age prevalence, with peak incidence first in the mid-second to early-third decade of life and second peak incidence between the late-fourth to seventh decade of life.

Sex

Women are affected more often than men (70% to 80% of patients are women) [2].

Microscopically, clusters of plasma cells in the interface of portal tracts and hepatic lobules are fairly characteristic for autoimmune hepatitis [5]. The salient features [6] include the absence of serologic markers of viral infection, elevated serum IgG and gamma-globulin levels (1-2-3x normal), and high serum titres of autoantibodies.

Subtypes

Type I

It is characterised by presence of positive ANA and ASMA [7] antibodies. Anti-actin antibodies may also be present. Gamma globulin levels are greatly elevated. This type occurs predominantly in elderly patients. HLA associations include DR3, DR4 and B8.

Type II

It is characterised by presence of Anti-LKM (anti-liver kidney microsomal antibody) and P450 IID6 antibodies. It occurs most commonly in young girls , and is very rare in adults. HLA associations include DR3, B14 and C4AQO.

Type III

This subtype occurs most commonly in middle-aged adults and is characterised by elevated levels of cytokeratin 8 and 18. Soluble liver-kidney antigens are also present. HLA association is unclear.

Sodium butyrate attenuated the injuries and prevented migration of Escherichia coli into the liver. [ncbi.nlm.nih.gov]

Treatment must be continued over several years and should not be discontinued too early in order to prevent recurrence. [drfalkpharma.com]

Prevention Autoimmune hepatitis cannot be prevented in most cases. Knowing the risk factors may help you detect and treat the disease early. Images References Czaja AJ. Autoimmune hepatitis. In: Feldman M, Friedman LS, Brandt LJ, eds. [ufhealth.org]

1. Krawitt EL: Autoimmune Hepatiti. N Engl J Med 354:54, 2006
2. McFarlane IG, Heneghan MA. Autoimmunity and the Female Liver. Hepatol Res. April 2004;28(4):171-176. [Medline]
3. Czaia AJ. Autoimmune Hepatitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th edition. Philadelphia. Pa: Saunders Elsevier;2010: Chap 88
4. Vento S, Cainelli F. Is there a role for viruses in triggering autoimmune hepatitis? Autoimmune Rev. Jan 2004;3(1):61-9
5. Czaja AJ:Autoimmune Liver Disease. Curr Opin Gastroenterol 23:255, 2007
6. Robbins and Cotran, Pathologic Basis of Disease. 8th ed. Pa: Saunders Elsevier. Chap 17. pg 855-56
7. Bogdanos DP, Invernizzi P, Mackay IR, Vergani D (June 2008). Autoimmune Liver Serology:Current Diagnostic and Clinical challenges. World J Gastroenterol. 14(21):3374-3387 doi 10.3748/wjg.14.3374 PMC 2716592 
8. Oettinger R, Brunnberg A, Gerner P, Wintermeyer P, Jenke A, Wirthe S. Clinical features and biochemical data of Caucasian children at diagnosis of autoimmune hepatitis. J Autoimmune. Feb 2005;24(1):79-84 
9. Mann MP, Strassburg, CP (2011). Therapeutic Strategies for Autoimmune Hepatitis. Digestive Strategies. Basel, Switzerland. 29(4):411-5. 
10. Stephen J McPhee, Maxine A Papadakis. Current Medical Diagnosis and Treatment 2009. Pg 596