What is Recurrent Pyogenic Cholangitis?
Recurrent pyogenic cholangitis, or RPC, is a disease that causes repeated infections in the bile ducts system, which was first identified in Hong Kong in 1930. This condition is known by various names, like Oriental cholangiohepatitis, Hong Kong disease, Oriental infestation cholangitis, or hepatolithiasis. Though it’s commonly found in Southeast Asia, increased travel and population movement have made it a worldwide issue now.
RPC is known for causing numerous tiny stones within the bile duct, which can lead to swelling and narrowing of these ducts. Patients with this condition often suffer from recurring episodes of acute cholangitis, or infection in the bile ducts, which makes them more likely to experience complications than those with more typical forms of cholangitis because of the persistent nature of the disease.
Diagnosing RPC can be tricky as its symptoms are very similar to other bile duct infection causes. This is why a team of various specialists, including emergency doctors, gastroenterologists, surgeons, interventional radiologists, and histopathologists, is required to accurately diagnose and treat this condition.
This write-up aims to review the causes, population distribution, and workings of RPC. It also plans to detail the examination and potential complications and risks of RPC along with the role of the healthcare team in managing patients with this illness.
What Causes Recurrent Pyogenic Cholangitis?
The key element of this disease process is primary hepatolithiasis. This condition involves the formation of multiple stones inside the liver’s bile ducts, but not outside the liver. These stones are generally brown in colour and consist of calcium bilirubin, a compound produced when the process of bilirubin deconjugation increases.
The exact cause of this increase remains uncertain, but many factors might be involved. It’s thought that severe malnutrition could contribute, as it can cause deficiencies in specific enzymes such as D-Glucaro(1,4)lactone. This enzyme plays an important role in preventing too much bilirubin deconjugation. If this enzyme is lacking, bilirubin levels increase and more calcium bilirubin is produced, leading to the formation of these stones.
Another contributing factor that’s proposed is parasite infection. Long-term infection with common parasites such as Clonorchis sinensis and Ascaris lumbricoides may cause inflammation in the cells lining the bile ducts, weaken the body’s immune response, and make the body more susceptible to infection from gut pathogens. Gut pathogens such as E.coli, Proteus, and Klebsiella can move up into the bile ducts and lead to further complications. In fact, eggs from the Ascaris parasite have been found at the core of these stones, suggesting they may play a role in the stones’ formation.
Conditions like malnutrition and parasite infection are often linked with a lower socioeconomic status. This association is becoming less relevant as regions in East Asia improve their economies and access to healthcare. To sum it up, this condition occurs due to stones in the liver’s bile ducts, triggering a harmful cycle of frequent bacterial infection, ongoing damage to the cells lining the ducts, and persistent inflammation of the bile ducts.
Risk Factors and Frequency for Recurrent Pyogenic Cholangitis
Rare in the United States and the West, Recurrent Pyogenic Cholangitis (RPC) is most prevalent in populations from East and Southeast Asia. The disease has been reported to affect up to 30% of individuals in these regions, especially those with endemic helminthic infection. With improved healthcare, hygiene, and dietary changes in the 21st century, the rates of RPC have likely decreased.
The disease typically affects more women than men and is considered a risk for people over the age of 50. In a study conducted in Korea, the average age for a patient at diagnosis was 59 years, with 71% of patients being women and 29% being men. Interestingly, a majority of the patients (82%) in this study had stones in their intrahepatic ducts, indicating a close relationship with RPC.
Data from Taiwan further suggests that more than half of the patients might have stones in their intrahepatic bile ducts. In 70% of such cases, there was the coexistence of intra and extrahepatic stones. These stones, primarily composed of calcium bilirubinate, play a critical role in the development of RPC.
Signs and Symptoms of Recurrent Pyogenic Cholangitis
Recurrent pyogenic cholangitis (RPC) is a condition that can range in severity, from mild to life-threatening abdominal infection. Patients suffering from RPC commonly have an array of symptoms including high body temperature, shivering, yellowing of the skin (jaundice), vomiting, abdominal pain, general feeling of discomfort (malaise) and itchiness (pruritus). A common combination of symptoms is known as Charcot’s triad, which is fever, jaundice, and upper right abdominal pain. It’s important to note that Charcot’s triad isn’t always present in every patient.
Patients might also have a history of returning biliary drainage procedures or a procedure known as endoscopic retrograde cholangiopancreatography (ERCP). Additionally, a patient may be from or have lived in areas known for the prevalence of RPC, such as Taiwan, Hong Kong, or Japan. Thus, getting a detailed social history is crucial.
In advanced cases of RPC, patients may experience symptoms related to complications like acute pancreatitis, which leads to stomach pain that is centrally located, or bile duct perforation that may cause acute, intense abdominal pain. In situations where pyogenic cholangitis progresses to bile duct cancer (cholangiocarcinoma), patients might look very thin and feel generally unwell, accompanied by weight loss and other chronically-ill health effects.
Testing for Recurrent Pyogenic Cholangitis
Diagnosing Recurrent Pyogenic Cholangitis (RPC) involves a combination of routine and specialized tests alongside a thorough patient evaluation. A doctor will examine a patient closely and take a comprehensive medical history.
Next, a series of blood tests are required. A complete blood count can identify anomalies like a long-term, low-grade anemia or an increase in white blood cells. If the patient is seriously ill with infection, measurements of kidney function may show a rise in waste products like urea and creatinine. It’s common for liver function tests to reveal a congestion in the liver with raised levels of gamma-glutamyl transferase, alkaline phosphatase, and bilirubin. Pathogens like E.coli may be found in blood cultures taken from the patient.
Imaging plays a key role in understanding the disease. Ultrasonography, which uses sound waves to create images, is often the first step. An ultrasound scan of the abdomen may reveal an enlargement of the tubes within the liver and leading from it. It may also show whether there are gallstones present, although they can be tricky to spot because they vary in how well they show up on ultrasound. Look out for increased brightness around the liver’s portal vein too. We can also assess other related conditions using ultrasound. For instance, if the wall of the gallbladder is thick, it’s more likely that the patient has gallbladder inflammation and less likely they have RPC. Sometimes, the picture can be confusing if the tubes within the liver are enlarged, but the ones leading from it aren’t.
Computed tomography (CT), which uses X-rays to create detailed images, provides similar information to ultrasound but is less dependent on the operator and provides a clearer view of the other organs in the abdomen. The CT scan might find enlargement of the channels in and round the liver, dense gallstones, thickening of the wall of the bile duct, or a narrowing of the bile duct. In severe cases, air might be found in the bile duct, or there might be shrinkage of the liver tissue, most often in the section on the left side.
Magnetic Resonance Cholangiopancreatography (MRCP), an MRI technique that provides imagery of the bile and pancreatic ducts, is the best non-invasive method for diagnosing RPC. Along with the findings observable on a CT scan, MRCP can also more accurately visualize the presence of gallstones and bile duct narrowings that are common with RPC. A commonly seen sign on MRCP with this condition is the ‘arrowhead sign’, a reduction in the smaller offshoots of the bile ducts. MRCP might also spot a subtle bile duct cancer.
Lastly, an invasive procedure called an Endoscopic Retrograde Cholangiopancreatography is helpful in diagnosing and treating RPC, but we’ll talk about that more in the ‘Treatment/Management’ section.
Treatment Options for Recurrent Pyogenic Cholangitis
Dealing with Recurrent Pyogenic Cholangitis, also known as RPC, requires the combined effort of various medical experts. This includes emergency doctors, interventional radiologists, gastroenterologists, general or liver surgeons, and other healthcare professionals.
If a patient comes into the hospital with acute symptoms, the first step is to stabilize them. They may receive oxygen, fluids, clot prevention treatments, and broad-spectrum antibiotics to address potential infections. If the situation is severe, they may be admitted to an intensive care unit.
When medicines aren’t enough, doctors might need to relieve the blockage of the bile duct, a procedure known as biliary decompression. They can use an endoscope to get a detailed view of how bad the situation is and to provide immediate treatment. For instance, they might remove a bile stone, cut a muscle around the bile duct to stop it from contracting, or insert a stent to keep bile flowing. If certain parts of the bile duct are blocked, a percutaneous transhepatic cholangiography (PTC) might be a better option.
While biliary decompression provides immediate relief, it only buys time while the medical team plans more permanent treatments. If the problem is limited to a specific area, such as left part of the liver, a surgical procedure might be necessary to remove the affected part and rebuild the bile duct. In more extensive cases, a doctor could create a new passage for bile to bypass blocked ducts or even consider removing part of the liver. Gallbladder removal is also often performed in severe cases.
One study from India found that most patients who had surgery became and remained free of bile stones for at least three years, with only a small percentage experiencing a recurrence of the disease. Another study in North America also found lower recurrence rates among those who had surgery. However, these are major procedures with significant risks, so the decision is not taken lightly. In very rare cases, and in situations of widespread disease, a liver transplant might become an option.
What else can Recurrent Pyogenic Cholangitis be?
When dealing with recurring infections of the bile ducts, a condition also known as recurrent pyogenic cholangitis, doctors often consider a number of different possible causes. These could include:
- Cholangitis, which is inflammation of the bile ducts
- Primary sclerosing cholangitis, a long-term liver disease which slowly damages the bile ducts
- Cholecystitis, or inflammation of the gallbladder
- Choledocholithiasis, commonly known as gallstones in the bile duct
- Liver abscess, which is a pocket of pus that develops in the liver
- Pancreatitis, inflammation of the pancreas
- Acute hepatitis, a sudden inflammation of the liver
- Decompensated liver failure, a stage of liver failure where the damage cannot be reversed
- Cholangiocarcinoma, a type of liver cancer that starts in the bile ducts
- Biliary helminthiasis, a parasitic infection of the bile ducts
- Anastomotic stricture after biliary tract surgery, a narrowing of the bile ducts following surgery
These alternatives must be carefully considered by the medical team to ensure the right diagnosis and the most effective treatment plan.
Surgical Treatment of Recurrent Pyogenic Cholangitis
Cholangiocarcinoma, a type of cancer, is known to occur as a complication in recurrent pyogenic cholangitis (RPC). One research study from Hong Kong looked at whether patients with both RPC and cholangiocarcinoma had a worse prognosis than those with just cholangiocarcinoma. This was done using a ‘propensity score matching’ method. This method compared how well patients did after liver surgery (hepatectomy) when they either had or didn’t have a diagnosis of pyogenic cholangitis, which is another term for RPC (the study included a total of 143 people).
The study showed that having previously suffered from pyogenic cholangitis led to significantly worse outcomes for patients, in terms of both how long they lived without their disease progressing (disease-free survival) and their overall ability to survive (overall survival).
What to expect with Recurrent Pyogenic Cholangitis
Studies looking back in time have suggested that disease recurrence is fairly common, even with aggressive treatment efforts. Some data indicates that patients with recurrent pyogenic cholangitis (RPCholangiocarcinoma was observed in 5% of these patients, and 20% may eventually die from RPC and its related complications, some of which can be life-threatening.
Possible Complications When Diagnosed with Recurrent Pyogenic Cholangitis
RPC, or retained putative capsule, may cause several complications including:
- Pancreatitis – inflammation of the pancreas
- Acute kidney injury – sudden episode of kidney failure or kidney damage
- Septicemia – severe infection that spreads through the bloodstream
- Disease recurrence – the disease comes back after treatment
- Liver abscess (pyogenic) – pus-filled pocket in the liver
- Portal hypertension – high blood pressure in the liver’s portal vein
- Secondary biliary cirrhosis – liver disease caused by damage to the bile ducts
- Chronic liver disease – long-term liver damage that can lead to cirrhosis
- Cholangiocarcinoma – cancer that forms in the bile ducts
Preventing Recurrent Pyogenic Cholangitis
Understanding the risk factors is crucial for lowering the chances of getting RPC, a type of liver disease. People living in areas where the disease is common should know what symptoms to look out for and take steps to lessen risk factors they can control. For instance, eating a balanced and healthy diet can help an individual maintain a suitable weight, which can reduce the risk of getting the disease.
Infections caused by liver flukes, a kind of parasite, can be treated with specific medications to prevent long-term infections. This step can also contribute to reducing the risk of RPC. Lastly, if a patient is diagnosed with RPC, it’s important they get medical help early to prevent serious health complications.