The jaundice may fluctuate in a certain range, accompanied by itchy skin, weight loss, poor spirit.The ascites, oliguria and hepatic coma may occurr late due to liver failure.

• 少数病人抗核抗体及抗平滑肌抗体可为阳性，但抗线粒体抗体阴性，这可与原发性胆汁性肝硬化区别。