Primary intrahepatic cirrhosis (Hanot) syndrome refers to a group of symptoms caused by nonspecific inflammation and fibrosis of the intrahepatic bile ducts, leading to intrahepatic bile stasis, liver cirrhosis with nodular hyperplasia. It is now commonly referred to as primary biliary cirrhosis, also known as chronic nonsuppurative destructive cholangitis, cholangiohepatitis, biliary cirrhosis, or intrahepatic obstructive biliary cirrhosis. This syndrome may result from autoimmune abnormalities, leading to nonsuppurative destructive inflammation of the septal bile ducts and interlobular bile ducts, accompanied by bile duct proliferation, destruction, scar formation, disruption of liver lobule structure, regenerative nodule formation, and histological changes of cirrhosis with significant bile stasis.

bubble_chart Clinical Manifestations

Clinically, it is more common in female patients with an insidious onset. Generalized cutaneous pruritus occurs initially, followed by jaundice that progressively deepens to grade III. Bile stasis can lead to steatorrhea, clay-colored stools, osteoporosis, pathological fractures, bleeding, and other symptoms. Hepatosplenomegaly is prominent, along with cutaneous xanthomas.

bubble_chart Auxiliary Examination

Laboratory tests showed elevated serum bilirubin, predominantly direct bilirubin, along with increased alkaline phosphatase, cholesterol, and IgM. Anti-mitochondrial antibodies, smooth muscle antibodies, and anti-nuclear antibodies were positive. Albumin levels were decreased, while gamma-globulin levels were elevated.

bubble_chart Diagnosis

Liver biopsy shows characteristic pathological changes of sexually transmitted disease. The disease can be diagnosed when extrahepatic obstructive jaundice and secondary biliary cirrhosis are excluded.

This disease can be treated with immunosuppressive therapy using adrenal corticosteroids or azathioprine. Cutaneous pruritus can be managed with antihistamines or cholestyramine, and aluminum hydroxide may also be tried. Other symptomatic treatments and prevention of complications such as upper gastrointestinal bleeding, hepatic encephalopathy, and ascites can also be performed.