Guide
Vaishing bile duct syndrome (VBDS) refers to the pathological process caused by a variety of factors, which destroys the structure of the intrahepatic bile duct tree and causes the focal or diffuse disappearance of the hepatobiliary duct, resulting in clinical cholestatic syndrome. Prevention and early diagnosis are extremely important due to its progressive process and potentially fatal outcomes. The etiology, clinical manifestations, diagnosis, and treatment of VBDS are summarized here for reference.
pathogen
The exact etiology and pathologic mechanism of VBDS are not well understood, but the possible causes include congenital malformations, developmental, hereditary, idiopathic, immune, infectious, ischemic diseases, and drugs (Table 1). Although uncommon, VBDS has been reported with a variety of drugs, including nonsteroidal anti-inflammatory drugs, antibiotics, antivirals, antipsychotics, lipid-lowering drugs, and sex hormones.
Table 1 Etiology of VBDS
Clinical presentation
The clinical presentation and course of VBDS are highly variable and depend on available treatments. Onset of disease can be rapid, such as acute cell rejection, or gradual, such as primary biliary cirrhosis.
VBDS typically presents with fatigue, anorexia, abdominal pain, and weight loss, and may present with jaundice, pruritus, xanthoma, and mild steatorrhea. As the history prolongs, the bile ducts gradually disappear, causing cholestasis. After several years of lesion development, hepatocyte loss, pseudolobular formation, peripheral manifold fibrosis, and finally cirrhosis of the liver, which may include spider angioma and splenomegaly, and ascites, edema, and hepatic encephalopathy in the advanced stage. In drug-related VBDS, hepatitis, cholangitis, and, less commonly, inflammatory pseudotumors may occur in some patients.
diagnosis
Diagnosis of VBDS is based primarily on clinical findings, laboratory tests, and liver biopsy.
Patients may have markedly elevated levels of alkaline phosphatase and γ-glutamyl transpeptidase, as well as direct or conjugated hyperbilirubinemia and mild aminotransferases. Because the disease is characterized by injury to the small bile ducts, imaging is usually undiagnostic, but extrahepatic biliary obstruction must be ruled out. Liver biopsy is necessary to confirm the diagnosis of VBDS, confirms ductal reduction, and may reveal an underlying cause. There are usually two potential outcomes: progressive, irreversible bile duct disappearance leading to widespread bile duct reduction and biliary cirrhosis, or gradual regeneration of the bile duct epithelium leading to clinical recovery.
treat
For the treatment of VBDS, the primary lesion should be considered first, mainly with corticosteroids, supplemented with vitamins, immunotherapy, and traditional Chinese medicine. Because there is no treatment to induce regeneration of bile ducts after they have disappeared, treatment is usually based on supportive care and symptom control. Ursodeoxycholic acid has the effects of immunomodulation, protection of liver cells, and removal of fat-soluble bile salts, which can relieve symptoms, but symptoms reappear after stopping the drug, and are relieved again with reuse. Immunosuppression may be one of the treatment options for VBDS, however, controlled trials are needed before it can be used as an effective treatment. Patients with biliary cirrhosis or advanced disease should be considered for liver transplantation.
参考文献：1. Reau NS, Jensen DM. Vanishing bile duct syndrome. Clin Liver Dis. 2008 Feb; 12(1):203-17, x. doi: 10.1016/j.cld.2007.11.007.2. 邵志江. 胆管消失综合征研究[J]. 医学综述, 2008(02):285-287.3. 孙玥, 赵新颜, 贾继东. 胆管消失综合征病因学诊断及预后进展[J]. 肝脏, 2014,19(02):137-139.DOI:10.14000/j.cnki.issn.1008-1704.2014.02.019.
Yimaitong is a professional online doctor platform, and the mission of the platform is to "sense the pulse of the world's medicine and help China's clinical decision-making". Yimaitong has a series of products such as "Clinical Guidelines", "Medication Reference", "Medical Literature King", "Yizhiyuan", "eYantong" and "ePulse", which fully meet the needs of medical workers in clinical decision-making, obtaining new knowledge and improving scientific research efficiency.