Special Circumstances

Inactive cirrhosis [162,163]


• A subset of patients with cirrhosis has inactive disease characterized by the absence of inflammatory cells on liver biopsy and normal or near-normal serum aminotransferases.
• The diagnosis of AIH may be presumed in such patients based upon the clinical setting, the presence of serologic markers of AIH, and the absence of other causes of liver disease. Such patients may be at increased risk for the development of corticosteroid-related side effects while the benefit of treatment is uncertain.
• Treatment should not be withheld from patients with decompensated cirrhosis who have active disease.
• The response may be excellent even in those who have already experienced bleeding from esophageal varices or who have significant ascites.
• Many patients respond when treatment is initiated, and the 10-year survival for treated patients, including those with cirrhosis, exceeds 90 percent
Pregnancy [164,165]


• Women appropriately treated for autoimmune hepatitis may have successful pregnancies.
• Usual therapy consists of corticosteroids and/or azathioprine, both of which are probably safe during pregnancy.
• Cessation of therapy during pregnancy in such patients has been associated with relapse of the disease.
• Pregnancy in women with AIH has been associated with an increased risk of prematurity, low birth weight and fetal loss [166,167].
• Patients need to be monitored carefully during pregnancy and several months post-partum because of the risk of flares in disease activity
Coexisting hepatitis C [168]
• In the rare instances in which autoimmune hepatitis is accompanied by chronic hepatitis C, treatment should first be directed toward autoimmune hepatitis because of the danger of exacerbating autoimmune hepatitis with interferon.
• Although this approach may result in raising viral levels of hepatitis C, it is the safer initial strategy.

Patients who are not treated
• Patients in whom treatment is not initiated should be monitored carefully, including performance of repeat liver biopsies for evidence of disease progression.
• Rebiopsy such patients no longer than two years after diagnosis, although the optimal management of such patients is unclear.
Treatment adverse effects
o Cushingoid features, acne, and hirsutism develop in 80% of patients after 2 years of prednisone-based therapy.
o Osteoporosis with vertebral compression, diabetes, cataracts, severe emotional lability, and hypertension may develop in patients who are treated with prolonged courses of high-dose prednisone.
o Premature treatment withdrawal is justified in patients who develop intolerable obesity, cosmetic changes, or osteoporosis.
o Azathioprine can function as a steroid-sparing agent. The authors have had great success and minimal drug-related adverse effects using a regimen of prednisone 10 mg/d plus azathioprine 50 mg/d.
o Patients should be co-treated with calcium and vitamin D in order to prevent the development of steroid-induced osteoporosis.
o Regular exercise should be encouraged.
o Bone densitometry performed every 1-2 years should be used to monitor patients.
o Signs of early osteoporosis may warrant the institution of treatment with alendronate.
o Azathioprine therapy can be complicated by cholestatic hepatotoxicity, nausea, vomiting, rash, cytopenia, and pancreatitis. These complications occur in fewer than 10% of patients treated with azathioprine at 50 mg/d.
o Hematologic malignancy has been reported in patients undergoing treatment with azathioprine; however, the risk of malignancy is thought to be low in patients with autoimmune hepatitis who are treated with low

FOLLOW UP
FOLLOW UP FOR PATIENT ON THERAPY [148, 158]
• If patient is on therapy get serum aminotransferases and circulating globulins (total or gamma globulin, or both, with or without IgG) every 1- 2 months to monitor response to therapy
• Consider a liver biopsy after aminotransferases are normal for atleast a year or approximately two years after presentation
• Patients receiving prednisone should undergo eye examinations for cataracts and glaucoma periodically during treatment.
• Asymptomatic patients on longterm corticosteroid treatment should be monitored for bone disease by annual bone mineral densitometry of the lumbar spine and hip.
• Patients receiving azathioprine in any dose should be monitored for leukopenia and thrombocytopenia
FOLLOW UP TREATMENT FOR PATIENT NOT ON THERAPY
• If patient not a candidate for treatment, liver biopsy every 2 years should be done [148]

FOLLOW UP AFTER REMISSION
• The threat of relapse is always present so that regular monitoring of transaminases is important indefinitely [148]

SURVEILLANCE
• Patients with cirrhosis due to AIH should undergo surveillance with a right upper quadrant ultrasound and alfa fetoprotein level every 6 to 12 months [148]


PROGNOSIS
• The prognosis of autoimmune hepatitis depends primarily on the severity of liver inflammation. Patients with a severe initial presentation tend to have a worse long-term outlook than patients whose initial disease is mild. Similarly, the inability to enter remission or the development of multiple relapses, either during therapy or after treatment withdrawal, implies a worse long-term prognosis [147].
• HLA status reflects treatment outcome. As an example, HLA DR3-positive patients are more likely to have active disease and are less responsive to therapy than patients with other HLA types. These patients also are more likely to require liver transplantation at some point.
• Spontaneous resolution of disease is observed in 13-20% of patients, regardless of the inflammatory activity. This is an unpredictable event.
• Hepatocellular carcinoma (HCC) is less common in patients with autoimmune hepatitis–induced cirrhosis than in cirrhosis caused by other factors; however, HCC is not a rare event in autoimmune hepatitis.
• Without therapy, most patients die within 10 years of disease onset [158]

PATIENT EDUCATION


• No specific diet has been shown to improve the outcome in patients with autoimmune hepatitis. The best advice is to eat a normal healthy and balanced diet while avoiding becoming obese, as fatty liver may be associated with obesity and may complicate the autoimmune hepatitis.

• Alcohol should be avoided since it can cause fatty liver and other liver damage. All types of alcoholic beverages can be harmful to the liver. Patients with liver disease may worsen even with small amounts of drinking.

• Exercise is good for your overall health and is encouraged, but has no clear effect on autoimmune hepatitis.

• Many drugs require metabolism by the liver. Thus, it is always best to check with your doctor or pharmacist before starting a new prescription. As a general rule, unless your liver is already scarred, most drugs are safe.

• An important possible exception is acetaminophen (Tylenol), the maximum dose of which should not be more than 2 grams (in divided doses) per 24 hours. Sometimes lower doses of medications are indicated in patients with liver disease.

• Allergic reactions to drugs may occur in patients with liver disease as they can in patients without liver disease.

• Although many claims about herbal medications have been made (particularly on the internet), none have been proven to improve outcomes in patients with autoimmune hepatitis, and some have been associated with serious liver toxicity.