This disease is a chronic progressive cholestatic liver disease of unknown cause, possibly related to autoimmunity.

bubble_chart Diagnosis

Medical history and symptoms

This disease often coexists with other immune-related conditions such as rheumatoid arthritis, Sjögren's syndrome, scleroderma, and chronic lymphocytic thyroiditis. It is more common in middle-aged women, with an insidious onset and slow progression. Early symptoms are mild, and patients generally remain in good condition, with no significant decrease in appetite or weight. About 10% of patients may have no symptoms at all. For patients with unexplained chronic progressive obstructive jaundice, especially those accompanied by steatorrhea, a detailed understanding of the predisposing factors and disease progression is necessary, along with checking for the presence of other immune-related diseases. Attention should be paid to differentiating it from secondary biliary cirrhosis and other causes of cirrhosis presenting with jaundice.

Physical examination findings

Jaundice of the skin and sclera, with multiple scratch marks and desquamation. The liver and spleen are enlarged with a smooth surface and no tenderness.

Auxiliary examinations

Significant elevation of blood lipids, serum bile acids, conjugated bilirubin, alkaline phosphatase (AKP), and gamma-glutamyl transferase (GGT), among other micro-biliary enzymes. Transaminase levels are normal or mildly to moderately elevated (grade II). Anti-mitochondrial antibodies are positive in the blood, IgM is elevated, and prothrombin time is prolonged. Urinary bilirubin is positive, while urobilinogen is normal or reduced.

Imaging studies such as ultrasound, ERCP, CT, and PTC are used to assess the presence of intrahepatic or extrahepatic bile duct dilation and diseases causing extrahepatic obstructive jaundice.

Adequate rest is essential, along with a diet high in protein, carbohydrates, and vitamins but low in fat, with daily fat intake preferably kept below 40–50g. Supplement fat-soluble vitamins A, D, E, and K. Ursodeoxycholic acid at 600–700mg/day, taken for over 6 months, can improve clinical symptoms and laboratory findings. Corticosteroids such as prednisolone at 30mg/day orally should be reduced to 10mg/day after symptom improvement and continued for one year. Monitor for worsening bone disease and bacterial infections in advanced cases. Azathioprine and cyclosporine A are effective but should be used cautiously due to risks of nephrotoxicity and bone marrow suppression.