Autoimmune hepatitis (AIH) may have an atypical serum alkaline phosphatase elevation, antimitochondrial antibodies, histologic features of bile duct injury/loss, or cholangiographic findings of focal biliary strictures and dilations. These manifestations characterize the overlap syndromes. Patients can be classified as having AIH with features of primary biliary cholangitis, primary sclerosing cholangitis, or a cholestatic syndrome. The gold standard of diagnosis is clinical judgment. Histologic evaluation is a major diagnostic component. Treatment is based on algorithms; outcomes vary depending on the predominant disease component. Combination therapy has been the principal recommendation.
Key points
- •
Autoimmune hepatitis may have cholestatic laboratory and histologic features that resemble primary biliary cholangitis, primary sclerosing cholangitis, or a cholestatic syndrome.
- •
Histologic findings may include destructive and nondestructive cholangitis, portal edema, portal fibrosis, periductal fibrosis, and ductopenia.
- •
Serum alkaline phosphatase levels greater than 2-fold the upper limit of normal range, concurrent inflammatory bowel disease, antimitochondrial antibodies, and recalcitrance to corticosteroid therapy are key clinical manifestations.
- •
Evaluation should include histologic assessment and endoscopic retrograde or MR cholangiography.
- •
Treatment recommendations emphasize mainly combination therapy with prednisone or prednisolone, azathioprine, and ursodeoxycholic acid, and outcomes vary depending on the predominant disease component.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
