by What is Primary Sclerosing Cholangitis?
Primary sclerosing cholangitis (PSC) is a rare chronic disease characterized by fibrosis and inflammation of the bile ducts within the liver. The bile ducts are progressively damaged over time, which can ultimately lead to cirrhosis and liver failure if left untreated. PSC most commonly occurs in men in their 30s and 40s and is often associated with inflammatory bowel disease.
Causes and Risk Factors of PSC
While the exact causes of PSC are unknown, genetic and autoimmune factors are believed to play a role. Genetic risk factors include the HLA-DR8 and HLA-DRB1*1301 genes which may make some individuals more prone to developing an abnormal immune response. Environmental triggers like infections, medications, or toxins are also thought to potentially contribute to onset of the disease in genetically susceptible individuals. Having inflammatory bowel disease, particularly ulcerative colitis, significantly increases the risk of developing PSC as well. Men have higher susceptibility compared to women.
Symptoms of PSC
The classic symptoms of Primary Sclerosing Cholangitis include fatigue, itching, jaundice, dark urine, and pale stools. As the disease progresses and more of the bile duct system becomes damaged, symptoms may worsen or new ones may emerge such as abdominal pain, nausea, vomiting, or weight loss. Advanced disease can lead to complications including liver cancer, esophageal varices, or portal hypertension which cause bleeding. Many people are asymptomatic at first and PSC may be detected incidentally during testing for something else.
Diagnosis of PSC
If symptoms or bloodwork indicate possible liver disease, the next tests are often blood tests to check liver function and imaging studies of the bile ducts. Endoscopic retrograde cholangiopancreatography (ERCP) allows visualization of the bile ducts and potential biopsy or dilation. Magnetic resonance cholangiopancreatography (MRCP) is non-invasive and also shows the ducts clearly. Liver biopsy may be done for abnormalities on other tests or when cancer is suspected. Definitive diagnosis requires characteristic changes seen on cholangiograms and exclusion of secondary causes that mimic PSC.
Treatment and Monitoring of PSC
Currently there is no cure for PSC, but treatments aim to reduce symptoms, slow disease progression, prevent complications, and improve liver transplantation candidacy. Ursodeoxycholic acid is often prescribed to help drain bile from ducts and lower cholesterol levels in bile. It has shown some effectiveness in improving biochemical markers of disease activity and delaying onset of symptoms in some people. Antibiotics may be used for dominant strictures (narrowed areas). Those with associated inflammatory bowel disease are often given medications for that condition. Liver transplants are the only option once cirrhosis or liver cancer develops, so close lifelong monitoring is needed. Monitoring involves regular blood tests and imaging to assess for new issues early, when treatment may be most effective.
Prognosis and Special Considerations
While the course of disease varies between individuals, about 50% of people diagnosed with PSC will develop cirrhosis within 10-20 years if left untreated. Annual risks of developing complications increase as disease progresses. However, appropriate treatment and close monitoring can help manage symptoms and improve overall prognosis greatly in many cases. Fatigue is a commonly reported issue, so energy conservation strategies and support groups can help. Due to increased risk for colon cancer as well as gallbladder and small bowel cancers in some cases, regular screening endoscopies are important. PSC is also associated with inflammatory arthritis in around 10% of patients. With multidisciplinary care and advancements in therapies, prognosis for PSC patients continues improving overall.
Liver Transplantation for Advanced PSC
Once advanced cirrhosis, liver failure, or liver cancer develops, liver transplantation becomes the only treatment option to remove the diseased liver. Success rates for PSC transplants are excellent and similar to survival for other causes of end-stage liver disease when performed at hospitals with extensive experience. But PSC disease process can recur about 10-30% of the time in the new organ. For this reason, lifelong immunosuppressants are required post-transplant to prevent rejection while monitoring for disease recurrence. Early referral for transplantation assessment helps determine candidacy and placement on transplant waitlists when needed. Having ulcerative colitis also elevates risks after transplant, including of colon cancer, so close attention is given to colon symptoms and screening as well. With continued improvements in transplant medicine, patients with PSC can expect good long term outcomes when transplantation is needed.
In summary, primary sclerosing cholangitis is a chronic disease affecting the bile ducts in the liver that can lead to cirrhosis and liver failure without treatment. While there is no cure currently, proper medical management focusing on symptom relief, slowing disease progression, and preventing complications can provide good long term prognoses for many individuals. Close monitoring and consideration of liver transplantation when advanced stages develop helps optimize patient outcomes. Greater understanding of the underlying disease mechanisms driving PSC continues propelling development of novel therapeutic targets and management approaches to improve quality of life for those living with this condition.
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1. Source: Coherent Market Insights, Public sources, Desk research
2. We have leveraged AI tools to mine information and compile it
Ravina Pandya, Content Writer, has a strong foothold in the market research industry. She specializes in writing well-researched articles from different industries, including food and beverages, information and technology, healthcare, chemical and materials, etc. With an MBA in E-commerce, she has an expertise in SEO-optimized content that resonates with industry professionals.
