Autoimmune hepatitis is liver inflammation due to immune system attacking hepatic cells with known or unknown reasons. It is a rare disease that is often associated with other autoimmune diseases including Grave’s disease, type 1 diabetes, lupus, Sjogren’s disease, rheumatoid arthritis, Crohn’s disease, ulcerative colitis, autoimmune thyroiditis and vitiligo. Autoimmune hepatitis is fourfold in females than in males with a genetic predisposition that might be triggered by environmental factors such as infections and drugs.
Autoimmune hepatitis has varied clinical presentations with being asymptomatic to presenting with acute liver failure. When presenting with symptoms, the patients show signs of fatigue, arthralgia, anorexia and if progressed further, it will show signs of cirrhosis. The diagnosis of autoimmune hepatitis is based on collection of clinical and laboratory findings such as symptoms along with elevated ALT/AST, increased levels of gamma globulins and IgG antibodies, presence of antinuclear antibodies, smooth muscle antibodies and liver/kidney microsomal antibodies along with histologic findings of interface hepatitis on liver biopsy. The diagnosis is confirmed provided other chronic hepatic diseases such as viral hepatitis, drug induced hepatitis, alcohol induced hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis are excluded.
The treatment of autoimmune hepatitis is directed at reducing the inflammation and damage caused by the immune system, which needs suppressing the immune system with the help of immunosuppressant drugs. The treatment is quite successful at suppressing the immune system with corticosteroids. Corticosteroids that are mostly used are high dose prednisone monotherapy, combination of prednisone and azathioprine or combination of budesonide and azathioprine.
What Is The Prognosis For Autoimmune Hepatitis?
The outcome of autoimmune hepatitis is extremely good and there is remission of the disease in about 80% of the cases after 1-2 years of treatment. The prognosis of autoimmune disease is very good in patients who are responsive to treatment with achievement of remission and 10-year survival rate in about 84-94% patients. Generally, patients require lifelong maintenance therapy as relapse ensues in about 80% patients within 3 years of withdrawal of therapy. Relapse shows elevated levels of AST/ALT as well as gamma globulin and IgG. Treatment is restarted with prednisone and azathioprine, with tapering of prednisone. The long-term maintenance therapy with azathioprine 50-100 mg daily monotherapy has reduced the possibility of long-term side effects of corticosteroid treatment. Treatment failure is seen in about 7% cases.
The patients with established liver cirrhosis in autoimmune patients prognosis is conflicting as some studies show 10 year survival rate and other studies show 10 year survival rate reduced to 64%. This needs further validation.
Although, most of the cases are managed well with corticosteroid therapy and there is no need for liver transplantation; however, it is required in some cases of acute liver failure, decompensated cirrhosis with end stage liver disease and hepatocellular carcinoma. 10-15% autoimmune hepatitis patients require liver transplantation (only 5% cases of liver transplantation are autoimmune hepatitis patients) and rest are managed with corticosteroid therapy. The prognosis is also good for patients with liver transplantation with 75% survival rate at 8 years. Recurrence of autoimmune hepatitis is seen in 20-30% of transplanted cases and is usually seen with the discontinuation of prednisone therapy. Immunosuppression regimen followed in liver-transplanted patients is a combination of calcineurin inhibitor (tacrolimus) with prednisone. Remission can be achieved with optimization of calcineurin inhibitors and reintroduction of prednisone and its continuation at low doses. These patients have comparable prognosis with patients of non-recurrence of autoimmune hepatitis in transplanted cases.
Monitoring of Autoimmune Hepatitis
Regular monitoring is imperative in autoimmune hepatitis patients with a follow up on live panel (ALT/AST levels as well as bilirubin levels), blood tests for complete blood count as well as gamma globulin and IgG levels prior the treatment, during the treatment as well as after treatment withdrawal. After the treatment withdrawal, monitoring is done (to ensure there is no recurrence of autoimmune hepatitis) every month for 3 consecutive months followed by monitoring at 6-12 months and if normal levels are maintained then yearly for life.
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