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Cholangitis

Disease Details

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Description: Cholangitis is an inflammation of the bile ducts, often caused by a bacterial infection that can lead to serious complications if not promptly treated.
Type: Cholangitis is not typically classified as a genetic disease. It is an inflammation of the bile ducts, often caused by bacterial infection, bile duct obstruction, or autoimmune conditions. There is no specific genetic transmission associated with cholangitis.
Signs And Symptoms: A person with cholangitis may complain of abdominal pain (particularly in the right upper quadrant of the abdomen), fever, rigors (uncontrollable shaking) and a feeling of uneasiness (malaise). Some may report jaundice (yellow discoloration of the skin and the whites of the eyes).Physical examination findings typically include jaundice and right upper quadrant tenderness. Charcot's triad is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever. This was assumed in the past to be present in 50–70% of cases, although more recently the frequency has been reported as 15–20%. Reynolds' pentad includes the findings of Charcot's triad with the presence of septic shock and mental confusion. This combination of symptoms indicates worsening of the condition and the development of sepsis, and is seen less commonly still.In the elderly, the presentation may be atypical; they may directly collapse due to sepsis without first showing typical features. Those with an indwelling stent in the bile duct (see below) may not develop jaundice.
Prognosis: Acute cholangitis carries a significant risk of death, the leading cause being irreversible shock with multiple organ failure (a possible complication of severe infections). Improvements in diagnosis and treatment have led to a reduction in mortality: before 1980, the mortality rate was greater than 50%, but after 1980 it was 10–30%. Patients with signs of multiple organ failure are likely to die unless they undergo early biliary drainage and treatment with systemic antibiotics. Other causes of death following severe cholangitis include heart failure and pneumonia.Risk factors indicating an increased risk of death include older age, female gender, a history of liver cirrhosis, biliary narrowing due to cancer, acute kidney injury and the presence of liver abscesses. Complications following severe cholangitis include kidney failure, respiratory failure (inability of the respiratory system to oxygenate blood and/or eliminate carbon dioxide), abnormal heart rhythms, wound infection, pneumonia, gastrointestinal bleeding and myocardial ischemia (lack of blood flow to the heart, leading to heart attacks).
Onset: Cholangitis typically has an acute onset, meaning symptoms can develop rapidly over a short period.
Prevalence: The prevalence of cholangitis, particularly acute cholangitis, can vary depending on the population and the presence of risk factors such as gallstones and biliary tract obstructions. It is difficult to pinpoint a specific prevalence rate, but it is generally considered a relatively uncommon condition. More detailed epidemiological data might be available through specific medical research studies.
Epidemiology: In the Western world, about 15% of all people have gallstones in their gallbladder but the majority are unaware of this and have no symptoms. Over ten years, 15–26% will have one or more episodes of biliary colic (abdominal pain due to the passage of gallstones through the bile duct into the digestive tract), and 2–3% will develop complications of obstruction: acute pancreatitis, cholecystitis or acute cholangitis. Prevalence of gallstone disease increases with age and body mass index (a marker of obesity). However, the risk is also increased in those who lose weight rapidly (e.g. after weight loss surgery) due to alterations in the composition of the bile that makes it prone to form stones. Gallstones are slightly more common in women than in men, and pregnancy increases the risk further.
Intractability: Cholangitis can be challenging to manage, but it is not generally considered intractable. Acute cholangitis, particularly when associated with bile duct obstruction, often requires prompt medical and sometimes surgical intervention. Chronic cholangitis, such as primary sclerosing cholangitis, may require ongoing treatment to manage symptoms and complications, but it is not always intractable. The effectiveness of treatment can vary based on the underlying cause and the patient's overall health.
Disease Severity: Cholangitis is an inflammation of the bile ducts and varies in severity, ranging from mild to life-threatening. If untreated, severe cases can lead to sepsis, liver damage, and other complications. Prompt medical treatment is crucial.
Healthcare Professionals: Disease Ontology ID - DOID:9446
Pathophysiology: Cholangitis is an inflammation of the bile ducts, often due to bacterial infection. The pathophysiology typically involves an obstruction in the bile ducts, which leads to stagnation of bile. This stasis creates a favorable environment for bacterial overgrowth and ascending infection, commonly from bacteria such as E. coli, Klebsiella, and Enterococcus. The obstruction can be caused by gallstones, strictures, tumors, or biliary sludging. Infections can lead to increased pressure within the bile ducts, causing the release of inflammatory mediators and potentially leading to systemic inflammation and sepsis if untreated.
Carrier Status: Cholangitis does not apply to carrier status as it is not a hereditary or communicable disease in the manner of genetic or infectious carrier states. It is an inflammation of the bile ducts, usually caused by a bacterial infection, obstruction, or autoimmune conditions. Being a carrier is not relevant to cholangitis.
Mechanism: Cholangitis is an inflammation of the bile ducts which can impede bile flow and cause infection. Here's a breakdown of its mechanisms:

### Mechanism:
- **Obstruction**: The most common cause is bile duct obstruction, typically from gallstones, strictures, or tumors.
- **Infection**: Bacterial infection, most commonly by E. coli, Enterococcus, Klebsiella, and Enterobacter, can ascend from the intestine due to stasis or obstruction of bile flow.
- **Immune Response**: The inflammation triggers an immune response, leading to additional damage and potential sepsis if untreated.

### Molecular Mechanisms:
- **Inflammatory Mediators**: Pro-inflammatory cytokines such as TNF-alpha, IL-1, and IL-6 are released in response to infection or injury, promoting further inflammation and recruitment of immune cells.
- **Oxidative Stress**: Reactive oxygen species (ROS) and nitric oxide (NO) produced during the inflammatory response can cause cellular damage to the bile duct epithelium.
- **Bile Acid Toxicity**: Accumulation of bile acids can directly harm hepatocytes, causing cell death and fibrosis through pathways involving apoptosis and necroptosis.
- **Fibrosis**: Chronic inflammation leads to activation of hepatic stellate cells, which produce excess extracellular matrix proteins, contributing to fibrosis and scarring of the bile ducts.
- **Bacterial Toxins**: Pathogens may produce toxins and enzymes that exacerbate epithelial damage, promoting further inflammation and fibrosis.

Understanding these mechanisms is crucial for developing targeted therapies to manage and treat cholangitis.
Treatment: Cholangitis is an infection of the bile ducts. Treatment typically involves:

1. Antibiotics to treat the infection.
2. Procedures to drain the bile ducts, such as Endoscopic Retrograde Cholangiopancreatography (ERCP) or percutaneous drainage.
3. Addressing the underlying cause of the blockage, such as removing gallstones, treating strictures, or managing tumors.
4. Supportive care, including intravenous fluids and pain management.

In severe cases, surgery may be necessary. Timely medical intervention is crucial to prevent complications.
Compassionate Use Treatment: Cholangitis is an inflammation of the bile ducts, often caused by bacterial infection. While standard treatment typically involves antibiotics, drainage of the bile ducts, and sometimes surgery, there are several off-label and experimental treatments under investigation.

1. **Endoscopic Therapy:** Besides standard endoscopic retrograde cholangiopancreatography (ERCP) with biliary stenting, there are off-label uses of biodegradable stents and drug-eluting stents being explored.

2. **Anti-inflammatory medications:** Off-label use of anti-inflammatory drugs like corticosteroids may be considered to reduce severe inflammation, though their effectiveness varies.

3. **Antimicrobial therapy:** Novel antibiotics or combinations of antibiotics are under investigation to address resistant bacterial strains causing cholangitis.

4. **Biologic agents:** Experimental treatments include biologic agents that target specific pathways involved in inflammation, though these are still in early stages of research.

5. **Liver Transplantation:** In cases of severe, recurrent, or treatment-resistant cholangitis, liver transplantation might be an experimental or compassionate use option.

6. **Photodynamic Therapy (PDT):** An emerging experimental technique involving the use of light-sensitive drugs and laser treatment to reduce biliary obstruction and inflammation.

Always consult with a healthcare professional for the most current and personalized medical advice.
Lifestyle Recommendations: For cholangitis, particularly when managing chronic cases or preventing episodes, consider the following lifestyle recommendations:

1. **Healthy Diet**: Eat a balanced diet rich in fruits, vegetables, lean proteins, and whole grains. Avoid fatty, spicy, and overly processed foods that can exacerbate symptoms.

2. **Hydration**: Drink plenty of water to help maintain hydration and support overall health, including liver function.

3. **Limit Alcohol**: Reduce alcohol intake or abstain completely, as alcohol can further damage the liver and complicate cholangitis.

4. **Regular Exercise**: Engage in regular physical activity to support overall health, improve circulation, and help maintain a healthy weight.

5. **Monitor Symptoms**: Keep track of any symptoms and consult healthcare providers regularly for appropriate management and intervention.

6. **Follow Medical Advice**: Adhere strictly to any prescribed medications or treatments and attend all medical appointments for monitoring.

7. **Avoid Hepatotoxic Substances**: Stay away from medications or substances known to harm the liver, unless approved by a healthcare provider.

Regular follow-ups with a healthcare provider are crucial to monitor the condition and adjust treatments as needed.
Medication: Cholangitis is typically treated with antibiotics to combat bacterial infection. Common antibiotics used include:

1. Piperacillin/tazobactam
2. Ceftriaxone combined with metronidazole
3. Ciprofloxacin combined with metronidazole
4. Ampicillin/sulbactam

In severe cases, hospitalization and intravenous antibiotics might be necessary. Additionally, treatment may involve procedures to relieve bile duct obstruction, such as endoscopic retrograde cholangiopancreatography (ERCP).
Repurposable Drugs: Repurposable drugs for cholangitis might include ursodeoxycholic acid, which is typically used for primary biliary cholangitis but can also help in other forms. Antibiotics like ciprofloxacin and metronidazole can be repurposed for bacterial cholangitis.
Metabolites: Cholangitis is an infection or inflammation of the bile ducts. Metabolites related to cholangitis can include elevated levels of bilirubin, alkaline phosphatase (ALP), and gamma-glutamyl transferase (GGT) due to bile duct obstruction. Nan, in this context, stands for "not applicable or none," indicating the question about "nan" does not apply to the topic of cholangitis or its metabolites.
Nutraceuticals: For cholangitis, there are no well-established nutraceuticals specifically recommended for treatment or management. This condition, an inflammation of the bile ducts, typically requires medical interventions, such as antibiotics for bacterial infections, and sometimes procedures to relieve bile duct obstructions. Always consult a healthcare provider for appropriate diagnosis and treatment.
Peptides: For cholangitis:

- Peptides: Certain antimicrobial peptides, such as defensins and cathelicidins, may play a role in the immune response to bacterial infections, which are often implicated in cholangitis. Research is being conducted to explore therapeutic peptides that can target and neutralize pathogens.

- Nan: Nanotechnology is being explored for its potential in diagnosing and treating cholangitis. Nanoparticles can be designed to deliver drugs directly to the site of infection or inflammation in the bile ducts, potentially improving treatment efficacy and reducing side effects.