Latest news with #primarysclerosingcholangitis
Health Line
4 days ago
- Health
- Health Line
Primary Sclerosing Cholangitis (PSC): Diagnosing and Living With This Liver Condition
Primary sclerosing cholangitis (PSC) affects the liver and bile ducts. Symptoms and treatment vary from person to person. PSC is a rare liver disease that affects the bile ducts. It's progressive, which means it gets worse over time. Treatment includes taking certain medications, including antibiotics, undergoing procedures to address blockages, and — in cases of liver failure — receiving an organ transplant. Here's what you need to know about PSC, its symptoms, and the outlook with proper treatment. What is primary sclerosing cholangitis? The liver is an organ in your body that breaks down food into its essential nutrients (proteins, fats, etc.) and energy, vitamins, and minerals. It also works to remove toxins from the bloodstream and assists in the digestive process by making a fluid called bile. More specifically, the bile ducts in the liver are responsible for transporting digestive juices (bile) from the liver to the small intestine and gallbladder. Bile helps break down fats and fatty vitamins in these organs and further aids digestion. With PSC, inflammation causes damage in the form of scarring, also called fibrosis. The scarring narrows and eventually blocks the bile ducts. When the ducts are blocked, the bile cannot travel where it needs to go. This results in liver damage. How many stages of primary sclerosing cholangitis exist? There are four stages of PSC: Cholangitis or portal hepatitis: With cholangitis, scarring is limited to the portal areas of the liver. Periportal fibrosis or periportal hepatitis: Areas of scarring extend beyond the portal areas but do not yet connect. Septal fibrosis, bridging necrosis (or both): Areas of scarring begin to connect to one another. Biliary cirrhosis: Scarring forms 'honeycomb' shapes and becomes more widespread. You may not have any symptoms or only mild signs in the beginning stages, so any symptoms you're experiencing do not necessarily relate to the stage of PSC. That said, symptoms tend to get more severe with each stage and continued damage to the liver. What causes primary sclerosing cholangitis? Doctors don't know exactly what causes PSC. And what causes it may be different for different people. What they do know is that: Some 70% of people who are affected are men, primarily young and middle-aged men. Around 80% of people with PSC also have inflammatory bowel disease. Several factors may increase your risk, like having certain viral or bacterial infections or a family history of the condition. What are the symptoms of primary sclerosing cholangitis? You may not have symptoms in the early stages of PSC. Some people may be asymptomatic or only have mild symptoms for the first few years. If you do have symptoms, they may come and go and get worse as the condition progresses. Possible symptoms include: fatigue itchy skin (pruritus) yellow skin and eyes (jaundice) fever, chills nausea abdominal pain (upper right) weight loss dark-colored urine, light-colored stools vitamin deficiencies enlarged liver or spleen If you're experiencing any concerning symptoms, make an appointment with a primary care doctor. They may refer you to a liver specialist called a hepatologist for more detailed testing and treatment. How is primary sclerosing cholangitis diagnosed? Signs of PSC may show up as abnormal results on liver blood tests. A more concrete diagnosis can be made after an liver MRI or a test called a cholangiography, where contrast dye is injected into the bile ducts and then viewed on an X-ray. How do you treat primary sclerosing cholangitis? To treat PSC, doctors will first personalize treatment by focusing on the symptoms a person is experiencing. The main goal of treatment is not to cure the condition but to slow its progression. Treatment options include: Medications: Your doctor may prescribe antibiotics to prevent infection or manage inflammation. Other medications include cholestyramine to help manage skin itching and bisphosphonates to protect bone mass and treat osteoporosis. Surgery: You may have endoscopic surgery to remove blockages in the bile ducts or widen narrowed ducts. Lifestyle changes: You may need to reduce or avoid your alcohol intake and quit smoking to further protect your liver. Eating a balanced, nutritious diet and taking supplements may also help address nutritional deficiencies. Vaccination: Your doctor may recommend getting vaccinated against both hepatitis A and B to provide extra protection for your liver. A liver transplant may be necessary in the later stages of PSC. This is usually around 10 years after diagnosis. Examples of later-stage complications might include: itching that is difficult to manage recurrent bacterial cholangitis end stage liver disease liver failure Liver transplants are generally effective in treating PSC, and many people go on to have a full, healthy life. However, it's also possible for PSC to come back, even with a transplant. Does the NHS cover primary sclerosing cholangitis liver procedures? The National Health Service (NHS) in the United Kingdom provides universal healthcare. The NHS generally pays for both inpatient and outpatient hospital care, which may include liver procedures and transplants. Still, there is no legislation or ' absolute right ' to specific care of any kind. It's important to check with your hospital for details on the coverage provided for you and your procedure. The average time a person waits for a liver transplant in the United Kingdom is between 5 and 7 months. This time may be shorter or longer depending on organ availability and your health status. Is primary sclerosing cholangitis covered by insurance plans in the United States? In the U.S., many insurance plans cover the basic treatments and prescription medications that might come with PSC. Of course, insurance plans vary, so it's important to know what your plan formulary allows. Most insurance companies also cover liver transplants, though it's important to check with your carrier to verify that coverage and your out-of-pocket costs. What's the long-term outlook for primary sclerosing cholangitis? Without treatment, people with PSC may experience: liver failure frequent infections certain cancers Both bile duct cancer (cholangiocarcinoma) and gallbladder cancer are two types of cancer that are more likely to occur in people with PSC. Again, this condition is progressive, so it tends to worsen over time. Treatment may slow the progression and treat symptoms, but a liver transplant is often necessary. The outlook for people who have undergone a liver transplant for PSC is favorable. Around 80% survive beyond 2 years after surgery with a good quality of life. Takeaway PSC is a rare, progressive liver disease that affects the bile ducts. Symptoms can vary in intensity and may include fatigue, itching, and yellowing skin and eyes. If you're experiencing any symptoms that concern you, make an appointment with a primary care doctor. They may refer you to a hepatologist for tests and treatments. There are various treatments for PSC, depending on the stage and symptoms you're experiencing. Some people with PSC may need a liver transplant around 10 years after their diagnosis. After a transplant, many people go on to have a good quality of life.
Medscape
24-06-2025
- Health
- Medscape
Liver Transplantation May Reduce Colorectal Neoplasia Risk
TOPLINE: In patients with primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), liver transplantation was associated with a 34% reduction in the risk for colorectal neoplasia. However, 21% of transplant recipients still developed neoplasia. METHODOLOGY: PSC affects 4%-8% of patients with IBD, increasing their risk for colorectal neoplasia. About 40% of patients with PSC undergo liver transplantation, but the post-transplant risk for colorectal neoplasia remains understudied. Researchers conducted a retrospective cohort study of patients with PSC and IBD from two sites in the United States, comparing outcomes between those who underwent liver transplantation with matched control patients who did not. All transplant recipients had at least one colonoscopy after the procedure. The primary outcome was the development of colorectal neoplasia, including colorectal cancer, low-grade dysplasia, or high-grade dysplasia. TAKEAWAY: The cohort included 979 patients (66% men; 83% with ulcerative colitis), of whom 320 underwent liver transplant and 659 served as controls; the mean age at IBD diagnosis was 32 years, at PSC diagnosis was 39 years, and at end of follow-up was 50 years. During follow-up, 21% of transplant recipients developed colorectal neoplasia compared with 26% of nontransplanted controls (P = .086). Liver transplantation was associated with a 34% reduced risk for colorectal neoplasia and a 44% reduced risk for both low- and high-grade dysplasia. Recurrent PSC in the transplanted liver was more prevalent in patients who developed neoplasia than those who did not (54% vs 38%; P = .017). Other predictors of dysplasia or cancer risk included older age (adjusted odds ratio [aOR], 1.02), diagnosis of ulcerative colitis vs Crohn's disease (aOR, 1.63), and histologic activity (aOR, 1.61). Undergoing chromoendoscopy at least once was associated with a higher likelihood of developing colorectal neoplasia (aOR, 1.71). IN PRACTICE: 'Careful profiling of PSC-IBD patients before and after [liver transplantation] offers an important opportunity to define the mechanism(s) of IBD-associated [colorectal neoplasia] in patients with PSC-IBD. This may also have broader implications for prevention of IBD-associated colon neoplasia,' the authors wrote. SOURCE: The study, led by Nayantara Coelho-Prabhu, MBBS, Mayo Clinic School of Medicine, Rochester, Minnesota, was published online in Clinical Gastroenterology and Hepatology. LIMITATIONS: The data were primarily derived from referral centers, possibly leading to an overrepresentation of patients with more severe disease. The study did not assess the cumulative burden of endoscopic and histologic activity over the entire course of IBD. The lack of information on the type of immunosuppression used post-transplantation may also affect the findings. DISCLOSURES: One author received grants from the National Institutes of Health (NIH), Leona M. and Harry B. Helmsley Charitable Trust, and the Chleck Family Foundation. Another author received a separate NIH grant. One author served on a scientific advisory board for Geneoscopy. This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
CBS News
14-06-2025
- Health
- CBS News
A father of 3 felt healthy. Then a routine screening found a rare, deadly illness.
Curtis Jackson was feeling fit and healthy in late 2020 when he decided to update his life insurance policy: He was in better shape than he'd been in a while, but he wanted to make sure his wife and three kids were taken care of if anything happened to him. Jackson, now 46, underwent an intensive physical, and everything seemed to be going well until he received a strange phone call. The call was focused on a questionnaire he had filled out. Jackson had truthfully marked that he did not drink alcohol. But his liver numbers were those of "a functioning alcoholic," he was told. It set off alarm bells. Jackson went to his primary care physician for another round of bloodwork. That doctor thought he had a problem with his gallbladder. Jackson went to have his gallbladder removed. During that operation, doctors found he had an unusual condition called primary sclerosing cholangitis, or PSC. The chronic, progressive disease affects the liver and inflames the bile ducts, causing liver damage. Patients eventually need a liver transplant. There's also a small but real chance of a deadly diagnosis: a dangerous cancer called cholangiocarcinoma. Early treatment and diagnosis can make a difference, so Jackson started undergoing two MRIs a year at the Mayo Clinic in Arizona to make sure he was cancer-free. He also had a regular test to check the progress of his PSC. In October 2024, Jackson had a nerve-wracking appointment where the technician scoping him seemed "very concerned." He felt healthy, but was worried by the appointment. "She didn't say that it was cancer, but she was hinting towards it," Jackson said. Curtis Jackson at home in April 2025. Mayo Clinic Three weeks later, Jackson and his wife received the news he had been dreading: The tests had diagnosed him with the deadly cancer. He would need to undergo intensive treatment to try to fight the illness. It felt like a repeat of when he had had the initial PSC diagnosis. "I was feeling fine ... and both times netted a result of something pretty serious," Jackson said. What is cholangiocarcinoma? Cholangiocarcinoma, or bile duct cancer, is considered a silent killer, according to Dr. Tanios Bekaii-Saab, the chairman of the division of hematology and medical oncology at the Mayo Clinic in Phoenix, Arizona. Bekaii-Saab was Jackson's oncologist. It tends to spread to other organs before symptoms appear, so most patients are diagnosed "too late to cure," Bekaii-Saab said. About 20% of people with PSC are diagnosed with cholangiocarcinoma, said Dr. Aqel Bashar, the director of the Mayo Clinic's Transplant Center in Phoenix, Arizona, who also treated Jackson. It's a rare disease, affecting about 1 in 100,000 people in the U.S., Bashar said. Not all cases are associated with PCS. Like many other cancers, it is on the rise, Bekaii-Saab said, with diagnoses of cholangiocarcinoma increasing more than 50% in the past decade. Fewer than 10% of the people diagnosed with cholangiocarcinoma live more than five years, Bashar said. The best way to treat cholangiocarcinoma is with a liver transplant, Bekaii-Saab said. But it can only be done if the disease is caught early, before the cancer spreads. Many cholangiocarcinoma patients are not eligible for a liver transplant. Thankfully, Jackson's case was caught early. He underwent three weeks of chemotherapy and radiation to kill as many cancer cells as possible. "Basically, I lived at Mayo Clinic. I would literally do work out of the parking lot, and then go and do radiation," Jackson said. During treatment, he lost about 30 pounds. But despite the odds, he remained optimistic that everything would work out. He even encouraged his wife to start a long-anticipated kitchen renovation. In the middle of the process, the family was told a liver was available. On March 6, he was wheeled into surgery. The Jackson family and their dog Lucy at home in April 2025. From left: Stetson Jackson, Gwyn Jackson, Rooney Jackson, Curtis Jackson and Kim Jackson. Mayo Clinic Surgery and recovery The surgery took about four hours. Six days later, Jackson was released from the hospital. Testing showed that he was cancer-free. For the next year, he will undergo weekly blood tests and have monthly check-ups. He will need close monitoring for the rest of his life, Bashar said, since his transplant needs to be monitored and doctors need to make sure his cholangiocarcinoma does not return. For now, Jackson is trying to focus on his home and family, he said. His routine is "pretty close to normal," though he still can't lift more than 10 pounds, and climbing stairs can be draining. He gained back much of the weight he lost in treatment, and his energy levels are close to where they were before the cancer diagnosis. "Things are definitely getting back to normal, which is really exciting. I wasn't expecting that so fast," Jackson said. "We might call it either lucky, or blessed, or whatever. I am just so thankful that I was able to bounce back so quickly." Curtis Jackson and his children hours after his transplant surgery. Jackson family The Jacksons are putting the finishing touches on the kitchen renovation, and planning to travel to North Carolina for vacation this summer. He also wants to plan family trips outside the country once he needs less frequent check-ups. His other priority is turning his story into something that can help others, and advocating for organ transplant and donation. "I have a new passion now," Jackson said. "I'm here because of these things. I would like to help this going forward and make sure people are learning the importance of donation."
