Finding the Patient With Primary Biliary Cirrhosis
Autoimmune hepatitis can occur in patients with primary PBC. Typical clinical and histologic findings of PBC include an elevated alkaline phosphatase level, positive AMA titer, bile duct injury, and cholestasis.
PBC often dominates the clinical expression of the two diseases, and establishing a diagnosis of an overlap between autoimmune hepatitis and PBC should be based on coexistence of the findings of PBC plus a serum alanine aminotransferase level that is elevated more than fivefold; an immunoglobulin G level elevated more than twofold; and the additional liver histologic features of autoimmune hepatitis, such as piecemeal necrosis or interface hepatitis.
Treatment of both diseases may be necessary, with corticosteroids added to management with ursodeoxycholic acid (UDCA). Patients with PBC and autoimmune hepatitis may respond less well to total therapy than to therapy for either disease alone.
The differential diagnosis of PBC includes other cholestatic disorders with ductopenia:
• Sclerosing cholangitis;
• Chronic hepatic allograft rejection;
• Biliary atresia;
• Idiopathic adult ductopenia;
• Sarcoidosis;
• Hodgkin disease; and
• Liver allograft hepatic artery occlusion.
Sclerosing cholangitis typically affects medium- and large-sized ducts, although an intrahepatic form affecting small interlobular bile ducts with periportal fibrosis and portal tract mononuclear inflammation also occurs. Sarcoidosis, which can result in granuloma formation, portal inflammation, and bile duct loss similar to PBC, tends to be scattered throughout portal areas and may have other manifestations, such as pulmonary disease. IgG4 cholangiopathy causes segmental inflammation and stenosis of medium and large bile ducts similar to sclerosing cholangitis. Cholestasis owing to drugs, pregnancy, and adult ductopenia should also be considered in the differential diagnosis of PBC.
Overlap Disorder
Autoimmune hepatitis can occur in patients with primary PBC. Typical clinical and histologic findings of PBC include an elevated alkaline phosphatase level, positive AMA titer, bile duct injury, and cholestasis.
PBC often dominates the clinical expression of the two diseases, and establishing a diagnosis of an overlap between autoimmune hepatitis and PBC should be based on coexistence of the findings of PBC plus a serum alanine aminotransferase level that is elevated more than fivefold; an immunoglobulin G level elevated more than twofold; and the additional liver histologic features of autoimmune hepatitis, such as piecemeal necrosis or interface hepatitis.
Treatment of both diseases may be necessary, with corticosteroids added to management with ursodeoxycholic acid (UDCA). Patients with PBC and autoimmune hepatitis may respond less well to total therapy than to therapy for either disease alone.
Differential Diagnosis
The differential diagnosis of PBC includes other cholestatic disorders with ductopenia:
• Sclerosing cholangitis;
• Chronic hepatic allograft rejection;
• Biliary atresia;
• Idiopathic adult ductopenia;
• Sarcoidosis;
• Hodgkin disease; and
• Liver allograft hepatic artery occlusion.
Sclerosing cholangitis typically affects medium- and large-sized ducts, although an intrahepatic form affecting small interlobular bile ducts with periportal fibrosis and portal tract mononuclear inflammation also occurs. Sarcoidosis, which can result in granuloma formation, portal inflammation, and bile duct loss similar to PBC, tends to be scattered throughout portal areas and may have other manifestations, such as pulmonary disease. IgG4 cholangiopathy causes segmental inflammation and stenosis of medium and large bile ducts similar to sclerosing cholangitis. Cholestasis owing to drugs, pregnancy, and adult ductopenia should also be considered in the differential diagnosis of PBC.
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