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Jul21
Autoimmune hepatitis – A Case Report
Abstract:
A rare case of cirrhosis of liver following autoimmune hepatitis is reported in a young female patient. The pathophysiology, clinical presentation and management of this condition are reviewed.
Key Words: Autoimmune, cirrhosis, hepatitis
Introduction:
Autoimmune hepatitis (formerly called autoimmune chronic active hepatitis) is a chronic disorder characterized by continuing hepatocellular necrosis and inflammation, usually with fibrosis, which tends to progress to cirrhosis and liver failure. The prominence of extrahepatic features of this disorder supports an autoimmune process in its pathogenesis. Because autoantibodies and other typical features of autoimmune disorder do not occur in all cases, a ‘broader’ more appropriate designation of this type of chronic hepatitis is idiopathic or cryptogenic.
Case Report
A 33 year old female patient came to us with history of amenorrhoea for last 6 months, gradual distension of abdomen for last 3 months, one episode of hematemesis 2 months back and scanty urination for 10 days.
One month prior to admission, the patient was diagnosed provisionally as a case of cirrhosis of liver and sclerotherapy was done for bleeding esophageal varices. Patient was on regular treatment with propranolol 40mg /day, Pantoprazole, Sucralfate and other supportivce drugs. Patient had no further hematemesis after sclerotherapy, but symptoms of progressive liver failure persisted. She is not a known case of DM, HTN, SCD, Pul, TB. There is no history of intake of heparotoxic drugs or joint pain. On examination, the patient was edematous with mild pallor, mild icterus, with a pulse rate of 68/min, regular, BP 110/90 mm Hg, CVS-NAD, chest-bilateral basal fine crepitions, abdomen-moderate ascites with engorged abdominal veins, the 24 hr urine output was 100ml.
Investigation
Hb% - 8.4%,
TLC – 7,900/mm3, DC-N 80%, E 6%, L-14%
RBS – 104m%
Urine Sugar Nil, Albumin - +
Sr. urea – 5-6%, Sr. creatinine 2mg%
Sr. Na+ - 100 mmol/lt, Sr. K+ - 2.0 mmol/lt
Sr. RA factor – Negative
HbsAg – Negative
LE cell – Negative
Anti nuclear antibody – positive (Odd ratro 1.64), (+ve>1.0)
Anti ds DNA – Borderline positive
LFT:
S. bilirubin 1` - 0.72mg%
S. bilirubin 30` - 2.11mg%
SGPT – 25IU/lt
SGOT – 62 IU/lt
Sr. Alk. Phosphate – 135 IU/lt
Sr. GGT – 12.2 IU/lt
Sr. Alb – 1.7 gm/dl
Sr. Protein – 6.5gm/dl
A: G ratio – 0.35
USG of abdomen – Ascites with splenomegaly, dilated portal vein
Chest X ray PA view – NAD
Needle biopsy of liver showed portal and periportal mononuclear infiltrate with areas of piecemeal necrosis and fibrosis.
The patient was diagnosed as a case of autoimmune cirrhosis of liver, started on oral prendisolone 30mg/day and other supportive treatment. The patient showed marked improvement after starting oral prednisolone. The condition of the patient was found to be stable on regular follow up since last six months.
Discussion:
Autoimmune hepatitis (AIH) – an inflammatory liver disease of unknown etiology characterized by suppressor – T cell defects and the production of autoantibodies directed against hepatocyte surface antigens. Two main types are recognized according to the presence of circulating autoantibodies.
Type I – After adults or childredn antinuclear antibodies (ANA) and / or
Antismooth muscle antibodies (SMA)
Type II – Affect children
Anti liver/kidney microsomal type I (LKM I) antibodies.
Clinical Features
Predominantly affects young and middle aged women, 25% present with acute hepatitis and features of an autommune disease e.g. fever, malaise, utticarial rash, polyrthritis, pleurisy or glumerulonephritis. The remainder present insidiously or are symptomatic and diagnosed incidentally with signs of chronic liver disease. Amenorrhoea is common.
Associations:
• Autommune thyroiditis
• Autoimmune hemolytic anemia
• Penicious anemia
• Diabetes mellitus (Type I)
• Ulcerative colitis
• Glomerulonephritis
• HLA AI, B8 and DR3 haplotype
Tests
• Abnormal LFT (AST)
• Hypergammaglobulinemia
• +ve for autoantibodies (ANA,SMA orLKM)
• Anemia TIC & TPC (Hypersplenism)
• Liver biopsy : Mononuclear infiltrate of portal and periportal areas with piecemeal necrosis, fibrosis and other features suggestive of cirrhosis.
• An ERCP should be performed to exclude primary sclerosing cholangitis if alkaline phosphatase is disproportionately raised.
Treatment
Immunosuppression (prednisolone, azathioprine )
Liver transplantation, but recurrence may occur
Prognosis : There is paucity of literature about autoimmune hepatitis among Indian population. The disease however shows variable natural history. Mild cases are marked by remissions and exacerbations, response to immunosuppression being excellent. Overall mortality without treatment is as high as 40%. Moderate to severe cases progress to cirrhosis of liver, hepatic failure and rarely hepatocellular carcinoma. In general, immunosupression remains the mainstay of treatment and treatment often slows down the disease process. Hence early diagnosis and institution immunosuppression is rewarding.
Conclusion
The diagnosis of autoimmune hepatitis requires a high degree of suspicion, thus many cases remain undiagnosed. All cases of hepatocellular failure where no obvious cause like viral hepatitis, alcoholism, ingestion of hepatotoxic drugs etc. are found should be investigated for autoimmune hepatitis.
References:
1. Krawitt E.L: Autoimmune hepatitis. N. Engl. J. Med. 334;897,1996.
2. Czaja A.J. et al. atommne hepatitis; Evolving concepts and treatment strategies. Dig. Dis. Sci. 40; 435,1995.
3. Czaja A.J. et al. Associations between alleles of the major histocompatibility complex and type I autoimmune hepatitis, Hepatology 25; 317, 1997.
4. Johnson P.J. et al. Meeting report; International autoimmune hepatitis group. Hepatology 18:998,1993.


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