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Citation:DOI:Assoc Prof Frank GaillardRevisions:see full revision historySystem:
- Primary sclerosing cholangitis
Primary sclerosing cholangitis is an uncommon inflammatory condition, which affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis.
The diagnosis can be made when there are classical imaging features in the correct clinical context, and secondary causes of cholangitis have been excluded 16.
What Complications Are Associated With Primary Sclerosing Cholangitis
PSC is a progressive disease, damaging the bile ducts and reducing the bile flow, ultimately leading to portal hypertension, cirrhosis, and liver failure.
Some cancers are associated with primary sclerosing cholangitis including gallbladder cancer, hepatocellular cancer and cholangiocarcinoma . The combination of inflammatory bowel disease and PSC increases the risk of developing cancers of the colon and rectum
What Is The Connection Between Psc And Ulcerative Colitis
About two-thirds of people with PSC have ulcerative colitis, a type of inflammatory bowel disease . People with PSC also may have another type of IBD called Crohns disease.
Its not entirely clear why so many people with PSC also have IBD. Researchers at UChicago Medicine are investigating the interaction between environmental triggers, the immune system and the gut microbiome to help understand the connection between the two diseases.
What is known is that people with PSC and ulcerative colitis have inflammation throughout their colon. They also have a higher risk for cancer in the colon, bile duct and gallbladder.
At the IBD Center and Center for Liver Diseases at UChicago Medicine, our specialists have extensive experience managing both conditions and can help people with PSC and IBD manage their special health risks.
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Looking After Yourself When You Have Primary Sclerosing Cholangitis
When someone has a long-term liver problem such as PSC, the livers ability to carry out its many functions may not be as good as in a healthy person. In particular, the livers ability to break down alcohol and medicines may be impaired, however this is often not a problem. If in doubt, check with your doctor.
Diet
People with PSC may not have any symptoms associated with fat malabsorption, while others develop symptoms of fat intolerance such as nausea, bloating and abdominal discomfort if they eat even moderate amounts of dietary fat. As the disease advances, the amount of fat excreted in the stools increases and they become bulky, pale and have a tendency to float in the toilet pan.
People with PSC vary in the amount of fat they can tolerate. Most find they are able to work out their tolerance level through trial and error by reducing their intake of higher fat foods. Stools become less frequent, darker and easier to flush away. If, however, steatorrhoea is still troublesome then specialist dietary advice will be needed in order to reduce dietary fat intake while keeping energy and protein levels relatively high.
Fats contain the fat-soluble vitamins A, D, K and E, and these may need to be supplemented. Oral supplements can be given but as the disease progresses it may be necessary to provide the supplements monthly by intramuscular injection.
As people with PBC and PSC are prone to bone disease, calcium supplements will usually be given alongside vitamin D.
Tiredness
Rapid Progression Of Primary Sclerosing Cholangitis Complicated With Ulcerative Colitis
Piotr Pardak
1Department of Internal Medicine, Institute of Rural Health, 20-090 Lublin, Poland
2Department of Clinical Endoscopy, Institute of Rural Health, 20-090 Lublin, Poland
3Department of Public Health and Social Medicine, University of Rzeszów, 35-959 Rzeszów, Poland
Abstract
Primary sclerosing cholangitis is a cholestatic condition with unknown etiology and long-standing, progressive course, leading to cirrhosis and requiring orthotropic liver transplant. In approximately 80%, primary sclerosing cholangitis is accompanied by inflammatory bowel disease, and in most cases the recognition of bowel disease precedes the diagnosis of primary sclerosing cholangitis. We describe a case of 22-year-old male diagnosed simultaneously with primary sclerosing cholangitis and ulcerative colitis, with a medical history suggesting uncommon prior development of the liver disease. Five months after the initial diagnosis, we observed advanced lesions of bile tree due to progression of primary sclerosing cholangitis, which led to the unusually fast necessity for the orthotopic liver transplant.
1. Introduction
2. Case Report
| Laboratory parameter |
The patients postoperative course was complicated by bleeding from the vascular anastomosis approximately one month after OLT and provided with surgical treatment. The patient is well 15 months after transplantation.
3. Discussion
4. Conclusion
Consent
Conflict of Interests
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What Is Vancomycin And Why Do Some Psc Patients Take It
Now, taking the antibiotic vancomycin for PSC is controversial. Vancomycin is normally prescribed to exterminate Clostridium difficileor C. diffwhich is a bacteria that causes diarrhea, stomach pain, fever, and even death. When doctors prescribe vancomycin for their PSC patients, it must be prescribed off-label since its not yet FDA approved for PSC.
But some doctors are confident that vancomycin can stop the progression of PSC AND treat inflammatory bowel disease symptoms.
Other doctors dont believe theres enough evidence supporting this, so they tend to prescribe their patients a drug called Ursodiol. .
To find out which was true for me, I agreed to stop Remicade and start Vanco. If, in six to eight weeks, Im out of my flare, we can pretty confidently say I have PSC. But, if in that time Im still sick, I likely dont have PSC.
Psst! I have an entire PSC Hub dedicated to vancomycin for PSC. To learn about its science and research, read patient stories, view best practices, and much more, check out the PSC Hub!
Can Primary Sclerosing Cholangitis Be Prevented
It is not possible to prevent PSC because the cause remains unknown. Liver damage and cirrhosis is often presumed to be caused by drinking too much alcohol, however PSC is not related to alcohol in any way.
Current evidence suggests that the disease may be triggered by an unknown bacteria or virus in people who are genetically programmed to get the disease. The common viruses known to cause hepatitis have not been associated with it.
The frequent occurrence of PSC in association with inflammatory bowel disease suggests that a common cause for both diseases may exist or that the inflamed colon allows toxins or infections to be absorbed into the body and this can cause the bile duct inflammation. The disease affects both genders, although two male patients are affected for every female patient. It can affect all ages, but it is most commonly found in young adults.
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How Do Doctors Make A Diagnosis Of Primary Sclerosing Cholangitis
PSC may remain silent for several years before symptoms arise. In patients with inflammatory bowel disease, the health care professional should have a high suspicion of the diagnosis because of the relationship between PSC and ulcerative colitis.
Depending upon how far the disease has progressed and the effect on liver function, physical examination may reveal an enlarged liver, tenderness in the right upper quadrant beneath the ribs, and an enlarged spleen. The skin may be jaundiced or yellow and there can be evidence of scratching due to intense skin itching. In cirrhosis with end stage liver disease, there may be bruising of the skin, a swollen abdomen due to ascites or fluid caused by decreased protein production and decreased blood flow through the scarred liver, gastrointestinal bleeding, and mental confusion because of elevated ammonia levels in the bloodstream.
Blood tests are helpful in assessing the liver and potential blockages within the bile ducts. These may include a complete blood count, INR/PTT , liver function studies including AST, and ALT to assess liver inflammation, alkaline phosphatase and bilirubin which measure the degree of bile blockage.
Imaging tests may include ultrasound to look at the liver structure and magnetic resonance cholangiogram, an MRI of the abdomen that can assess the bile duct structure of the liver.
Should infection occur, treatment may require antibiotics.
Characteristics Of Psc Associated With Ibd
While the presence of IBD with PSC appears to convey a specific IBD phenotype, the converse does not appear to be true. Microscopically, an early study noted no significant difference in the degree of histologic periductal fibrosis, periductal inflammation, portal edema or fibrosis in PSC vs. PSC-UC patients . Macroscopically, data conflict regarding the degree of intrahepatic and extrahepatic involvement in PSC-IBD vs. PSC alone . Clinically, PSC outcome measurements, including transplant-free survival rates, cirrhosis rates and mortality, did not differ significantly between PSC-IBD patients and those with PSC-alone . Notably, however, recent studies by Fevery and Weismüller report higher rates of liver-related morbidity and mortality in PSC-UC patients when compared to patients with PSC-CD or PSC alone. These data suggest the subtype of IBD may have an impact on the course of PSC disease progression .
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How I Felt After Taking Vancomycin For 6 Weeks
But after exactly six weeks, I felt a significant improvement in my ulcerative colitis flare! Suddenly I no longer needed to urgently run to the restroom. My abdominal pain vanished. I got my energy back. I didnt have to keep calling out of work.
I returned to my doctor at that time, my prednisone cheeks finally returning to their natural state. She was elated. She said I looked healthy. I hadnt felt this great in years.
Primary Sclerosing Cholangitis: What You Need To Know
- Most patients with PSC are men diagnosis usually occurs around 40 years of age.
- Inflammatory bowel disease increases your risk for developing PSC.
- A specialized test called an endoscopic retrograde cholangiopancreatography is often used to diagnose PSC.
- There are many treatment options for PSC, ranging from medication to surgery.
PSC occurs when the bile duct system becomes inflamed, then scarred and thickened , and ultimately obstructed. Usually a blockage or obstruction in the bile duct system causes the inflammation. Patients with PSC often have underlying conditions, such as inflammatory bowel disease .
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Recurrent Cholangitis And Dominant Strictures
Dominant strictures are defined by a diameter of 1.5 mm or less in the common bile duct or 1.0 mm or less in the common hepatic duct and are concerning for their propensity to cause recurrent cholangitis and to harbor or develop CCa.
Multiple modalities have been described for treating patients with biliary strictures, including percutaneous, endoscopic, and surgical modalities. ERCP is currently the most useful in managing strictures with balloon dilatations and stents. Endobiliary stents are typically reserved to patients who fail balloon dilatation. Not uncommonly, repeat ERCP is needed for recurrent strictures. Percutaneous interventions are also available but are falling out of favor given advancements in ERCP. Nevertheless, percutaneous transhepatic drainage remains a useful intervention in cases where endoscopic therapy is not available or technically feasible. Periprocedural antibiotic coverage is very important in PSC patients, especially in those previously instrumented or with recurrent cholangitis. Some patients who continue to suffer from recurrent cholangitis despite successful management of strictures benefit from rotating antibiotics to mitigate recurrent cholangitis episodes.
Axial CECT in a patient with advanced PSC-induced liver disease shows a dysmorphic liver with caudate hypertrophy and peripheral atrophy. The intrahepatic bile ducts are irregularly dilated, especially in the left lobe .
Fred F. Ferri MD, FACP, inFerri’s Clinical Advisor 2022, 2022
Why I Started Vancomycin And How It Led To My Psc Diagnosis
At the end of 2017, I was struggling. I was in a horrendous ulcerative colitis flare and dealing with abdominal pain, urgency, and chronic fatigue. Despite increasing Remicades dose and frequency, it wasnt helping anymore. Honestly, I was in so much pain that I was ready to talk about ostomy surgery.
However, my GIstrongly believing I had PSCmade an interesting suggestion. She recommended that I try vancomycin for a short period of time and see how my body responds. Ultimately, trying vancomycin would help us figure out if I had PSC or not.
How?
If I DID respond well to the medication, it meant I have PSC because the drug is doing its job by relieving me of my ulcerative colitis symptoms and lowering my elevated liver numbers.
If I DIDNT respond to the medication, it meant I didnt have PSC because theres nothing for the drug to act on.
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What Foods Should You Avoid If You Have Psc
Although there is no specific diet for people with PSC, we recommend a diet with plenty of fruits and vegetables, low-fat proteins and whole grains, specifically the Mediterranean diet. Your liver specialist may recommend other dietary changes that can help protect your liver. Coffee is also good for the liver and can decrease the risk of fatty liver and scarring.
Risk Of Malignancy In Psc
There is a high risk of malignancy in both PSC and IBD alone. However, their combination confers a significantly greater risk and requires shorter screening intervals for both CRC and hepatobiliary malignancy. Unfortunately, because of the relatively recent description of this phenotype, there is no strong evidence to inform malignancy screening guidelines. As a result, several of the screening recommendations are from guidelines describing PSC and IBD alone and are extrapolated to include screening in PSC-IBD patients.
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How Is Psc Treated
There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.
Search for a Clinical Trial
Clinical trials are research studies that test how well new medical approaches work in people. Before an experimental treatment can be tested on human subjects in a clinical trial, it must have shown benefit in laboratory testing or animal research studies. The most promising treatments are then moved into clinical trials, with the goal of identifying new ways to safely and effectively prevent, screen for, diagnose, or treat a disease.
Speak with your doctor about the ongoing progress and results of these trials to get the most up-to-date information on new treatments. Participating in a clinical trial is a great way to contribute to curing, preventing and treating liver disease and its complications.
Start your search here to find clinical trials that need people like you.
Clinical Characteristics Of Ibd Associated With Psc
There are a number of unique features of Ulcerative Colitis associated with PSC .6). The colitis is often mild, asymptomatic and runs a quiescent course. It is associated with rectal sparing and therefore not seen on rigid sigmoidoscopy is often more severe proximally and associated with backwash ileitis. It may be visible microscopically with a relatively normal macroscopic appearance and pouchitis is far more common after colectomy and ileo-anal pouch formation, occurring in up to 60% of PSC patients compared to 15% in patients with UC alone.
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What Are The Risk Factors For Primary Sclerosing Cholangitis
The cause of PSC is unknown but it may be an autoimmune disease.
PSC is more often seen in men in the 20-30 age range. There is an association with inflammatory bowel, disease, most often with ulcerative colitis and less commonly with Crohn’s Disease.
People with PSC who do not have inflammatory bowel disease, are more likely to be female and older.
In its early stages, PSC is asymptomatic . It is only when the person develops cholestasis because of the inability of bile to drain from the liver, do symptoms occur. These early symptoms include itching and fatigue. As the bilirubin levels in the blood become elevated, jaundice or a yellowish tinge to the skin, may occur. Since there may be liver inflammation, the person may complain of pain beneath the ribs in the right upper quadrant of the abdomen.
The inability of the bile ducts to adequately drain reduces the flow of bile and there can be sludge formation in the bile ducts leading to the risk of infection. This may cause fever and increased pain.
Because PSC is a progressive disease, symptoms may come and go over several months and years after the diagnosis is made.
Can Primary Sclerosing Cholangitis Be Misdiagnosed
Diagnosing PSC requires expertise, so its possible that you may have PSC for many years before it is accurately diagnosed. One reason is that you may have PSC without any symptoms just certain signs in your bloodwork. That is why it is important to go to a center like the one at UChicago Medicine that has extensive experience diagnosing and managing patients with liver diseases like PSC.
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Symptoms Diagnosis And Treatment
Primary sclerosing cholangitis is a rare and chronic disease of the liver that causes inflammation and narrowing of the bile ducts inside and outside of the liver. It’s not certain what causes PSC, although it is thought to be an autoimmune condition. PSC is not thought to be directly inherited, but it is thought to have a genetic component.
Bile is necessary for the digestion of fat and to carry waste out of the liver. PSC causes the bile ducts to narrow from scarring and inflammation, and bile begins to accumulate in the liver, which damages it. This damage eventually leads to scar formation and cirrhosis, which prevents the liver from performing its important functions. PSC can lead to a cancerous tumor of the bile ducts called cholangiocarcinoma, which occurs in 10 to 15% of patients.
PSC does progress slowly in most cases, but it can also be unpredictable and life-threatening. People with PSC can receive treatment to relieve symptoms and help them lead an active life.