Autoimmune hepatitis
Chronic autoimmune disease which causes acute and severe onset (more so than hepatitis B or C) with symptoms of fatigue, ill health, jaundice, malaise, anorexia, nausea, arthralgia and right upper quadrant pain.
It’s characteristic feature is the presence of autoantibodies in serum along with high levels of serum immunoglobulins and association with other AI diseases. Its typical manifestation is between 15-25 or 45-60 with an incidence of 1.7/100,000. Along with PBC and PSC, it is one of the three major autoimmune diseases of the liver.
Histology of the liver helps with diagnosis, key features include:
- Mixed inflammatory infiltrate centered on the portal tract
- With CD4+ T-cells, plasma cells and eosinophils
- Necrosis of periportal hepatocytes
- May progress to fibrosis and cirrhosis
Has two types, type 1, with titers of 1:80 or more of antinuclear, anti-smooth muscle, anti actin and anti-asialoglycoprotein receptor antibodies. Type 2 with elevations of anti-liver-kidney microsomal 1 antibodies and anti-liver cytosol 1 antibodies, without the elevation of the above antibodies.
Treatment is with corticosteroids. Begin with prednisolone (usually 20-30mg/day). If this does not cause biochemical markers to improve, consider progressing to Azathioprine + Prednisolone combination. As time progresses, look to find maintenance dose.