Introduction
Vanishing bile duct syndrome is a rare, acquired, serious form of chronic cholestatic liver disease. VBDS is not an independent disease but a pathological feature of the progressive destruction of intrahepatic bile ducts.
What Is Vanishing Bile Duct Syndrome?
VBDS is a rare, acquired, serious form of chronic cholestatic liver disease (a condition characterized by the defective transportation of bile from the liver to the intestine). Histologically, it is seen as severe bile duct destruction and usually manifests about one to six months after the onset of liver injury. VBDS is not an independent disease but a pathological feature of the progressive destruction of intrahepatic bile ducts. Therefore, the prognosis is variable and depends on the etiology of the injury to the bile duct. Drugs that cause VBDS are antibiotics like Amoxicillin, fluoroquinolones like Ciprofloxacin and Levofloxacin, macrolides like Azithromycin, antiviral drugs like Nevirapine, certain anticonvulsants, NSAIDs (non-steroidal anti-inflammatory drugs), etc.
Who Is at Risk of Vanishing Bile Duct Syndrome?
According to research, 95 percent of those affected by VBDS are women, with peak ages between twenty and sixty. In addition, the incidence of the disease is increasing.
What Are the Causes of VBDS?
VBDS can be associated with the following:
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Primary biliary cholangitis (a liver disease that harms the bile ducts).
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Infections.
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Congenital (due to developmental anomalies).
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Chromosomal associations like trisomies 17, 18, and 21.
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Ischemia.
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Adverse effects of drugs.
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Autoimmune disease.
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Allograft rejections.
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Hodgkin’s lymphoma (cancer originating from a type of white blood cell called lymphocytes).
What Are the Signs and Symptoms of VBDS?
More than 50 percent of the people were asymptomatic when diagnosed with this condition. Most consulted dermatologists for pruritus (itching of the skin) that persisted after pregnancy. However, the following symptoms were the most commonly observed:
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Persistent fatigue.
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Jaundice (yellowish discoloration of the skin and whites of the eyes).
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Anorexia (an eating disorder characterized by low body weight).
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Abdominal pain.
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Dyslipidemia.
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Unexplained weight loss.
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Persistent pruritus (itching of the skin).
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Malabsorption.
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Fat-soluble vitamin deficiencies.
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Elevated alkaline phosphatase levels.
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Elevated gamma-glutamyl transferase levels.
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Elevated conjugated bilirubin levels.
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Steven Johnson’s syndrome in severe cases (Steven Johnson syndrome is a rare, serious disorder of the skin and mucous membranes. It is usually seen as a reaction to certain drugs and can be seen as the flu, which can later progress to rashes and blisters).
How Is VBDS Diagnosed?
1. A thorough case history needs to be taken to understand if any drugs have been in prolonged use, about the use of over-the-counter drugs, or any other related information.
2. Physical examination - Jaundice (yellowish tinge in skin and the white of eyes) is most commonly observed. The patient might also complain of pruritus, unexplained weight loss, and a loss of hunger cues.
3. Laboratory investigations include blood tests and other liver function tests. Hyperbilirubinemia, along with elevated alkaline phosphatase levels, can be observed in liver diseases. An increase in gamma-glutamyl transpeptidase levels can confirm VBDS. Total immunoglobulin levels might be normal, but an elevated immunoglobulin M level can be observed. Antimitochondrial antibody levels also would be elevated.
4. Imaging tests, including ultrasonography of the biliary tree, are essential to rule out extrahepatic issues. In addition, computed tomography (CT) is done to understand organomegaly.
5. Adequate serological evaluation for autoimmune hepatitis and primary biliary cholangitis is to be done.
6. The final diagnosis can be established with a liver biopsy. A liver biopsy also helps determine the extent of the condition.
What Is the Treatment of VBDS?
The treatment of VBDS is based on the underlying reason. Therefore, the first treatment step is discontinuing the causative agent after identifying it, generally followed by clinical improvement. Alternative causes of liver injury include alcohol consumption and viral, autoimmune, and ischemic hepatitis.
Mild pruritus can be treated using antihistamines like diphenhydramine and hydroxyzine. The sedative effects of these drugs may be beneficial because pruritus is more common at night, causing sleep disturbances. More severe pruritus can be treated with cholestyramine, which binds and traps the pruritogenic in the intestine. Hyperlipidemia also requires therapy and responds minimally to HMG Coenzyme A inhibitors (statins), which are best to avoid.
Ursodeoxycholic acid is found to protect the epithelium against toxic bile salts. In addition, it stimulates hepatobiliary secretions, has oxidant therapy, enhances glutathione levels, and inhibits liver apoptosis, a prominent feature of liver diseases.
Corticosteroids are used in treating cholestasis and VBDS, but there is no evidence to prove they are beneficial; instead, they can worsen the secondary metabolic effects of end-stage liver disease. Therefore, liver transplantation may be an option in severe cases and for people with cirrhosis.
VBDS can slowly resolve on its own, while the main focus should be avoiding further injury. Careful attention to nutrition management (fat-soluble vitamins like A, D, E, and K; calcium and minerals) is essential in managing patients with this rare and challenging condition.
How Can VBDS Be Avoided?
Lifestyle modifications can help reduce the occurrence of VBDS, such as:
1. Alcohol Consumption: Avoiding or limiting alcohol consumption can help you stay healthy and prevent liver diseases.
2. Smoking: Quit or do not start at all. This lifestyle modification can keep our liver and other organs healthy.
3. Over-the-Counter Drugs: Avoid buying medications over the counter without a prescription from a doctor. The unnecessary use of drugs can cause harm to the liver and other organs of the body.
4. Immunization: Proper immunization can help prevent infections, improving the quality of life.
5. Eat Healthy: Eating healthy is the key to a healthy body. Include lots of fresh fruits and vegetables in your diet.
6. Regular Exercise: Keeping our bodies active helps maintain a healthy body. It enables us to sweat off toxins, making our body fit to live.
7. Others: Take precautions to avoid blood-borne or sexually transmitted diseases. To avoid cirrhosis, get proper hepatitis treatment. Maintain a healthy weight.
Conclusion
The prognosis of VBDS is variable and depends upon the causative agent of biliary epithelial apoptosis and the regenerative capacity of the biliary epithelium. In addition, various factors determine the prognosis of VBDS like the severity of the liver disease, the stage of the disease, performance status, comorbidities, alcohol consumption, pre-existing liver diseases, etc.
