The clinical presentation is variable, ranging from asymptomatic transaminase elevation to nonspecific constitutional symptoms, to advanced liver disease.
A chronic inflammatory disease of the liver, although 25% of cases present as acute hepatitis
Annual incidence of 1.4 cases/100,000; 3–4 times more common in women than in men
Wide age distribution, occurring in infants and octogenarians
27% 10-year survival in untreated patients
Drug-induced autoimmune hepatitis, reported with minocycline, nitrofurantoin, atorvastatin, and infliximab, has a more benign course than idiopathic autoimmune hepatitis.
Antinuclear antibodies (ANA): sensitivity, 32%; specificity, 76%; LR+, 1.3; LR–, 0.89
Anti-smooth muscle antibody (SMA): sensitivity, 16%; specificity, 96%; LR+, 4; LR–, 0.87
ANA and SMA: sensitivity, 43%; specificity, 99%; LR+, 43; LR–, 0.57
Diagnostic criteria have been developed (Table 26-7).
For a diagnosis of probable autoimmune hepatitis (6 points): sensitivity, 88%; specificity, 97%; LR+, 29; LR–, 0.12
For a diagnosis of definite autoimmune hepatitis (≥ 7 points): sensitivity, 81%; specificity, 99%; LR+, 81; LR–, 0.19
Table 26-7.Diagnostic criteria for autoimmune hepatitis. ||Download (.pdf) Table 26-7. Diagnostic criteria for autoimmune hepatitis.
|Variable ||Points1 |
|ANA or SMA ≥1:40 ||1 |
ANA or SMA ≥1:80 or
Anti-LKM ≥ 1:40 or
|IgG > ULN ||1 |
|IgG > 1.1 × ULN ||2 |
|Histology compatible with autoimmune hepatitis ||1 |
|Histology typical for autoimmune hepatitis ||2 |
|Absence of viral hepatitis ||2 |
|Interpretation: 6 points = probable autoimmune hepatitis; ≥ 7 points = definite autoimmune hepatitis |
Treatment is indicated for all patients with evidence of active inflammation, either by transaminase elevation or histology.
Prednisone alone, or prednisone and azathioprine are used to induce remission; occurs in 85% of patients
Many patients require maintenance therapy, most commonly with azathioprine.
Other immunosuppressive agents are used in patients who do not respond to or cannot tolerate the first-line treatments.
Liver transplant is often successful in patients with cirrhosis and decompensated end-stage liver disease, although autoimmune hepatitis can recur in the transplanted liver.