Overview
Primary sclerosing cholangitis (PSC) is a disease that affects the bile ducts, which carry bile from the liver to the small intestine. In PSC, inflammation causes scars within the bile ducts, leading to their hardening and narrowing. This eventually causes liver damage. A majority of people with PSC also have inflammatory bowel disease, such as ulcerative colitis or Crohn's disease. The disease progresses slowly in most cases, and can lead to liver failure, recurrent infections, and bile duct or liver tumors. The only known cure is a liver transplant, but it may recur in some patients. Care for PSC involves monitoring liver function, managing symptoms, and performing procedures to temporarily open blocked bile ducts. Early signs may include fatigue, itching, yellow eyes and skin (jaundice), and abdominal pain.
Understanding the disease
Symptoms
The symptoms of primary sclerosing cholangitis may include:
- Fatigue
- Itching
- Yellow eyes and skin (jaundice)
- Abdominal pain
- Fever
- Chills
- Night sweats
- Enlarged liver
- Enlarged spleen
- Weight loss These symptoms can appear as the disease progresses. It is important to see a doctor if you have severe, unexplained itching or extreme fatigue, especially if you have a history of inflammatory bowel disease such as ulcerative colitis or Crohn's disease.
Risk Factors
Risk factors for developing primary sclerosing cholangitis include age (the disease can occur at any age but is most often diagnosed between 30 and 40), sex (it occurs more often in men), and having inflammatory bowel disease, which is an umbrella term that includes ulcerative colitis and Crohn's disease. Additionally, people with Northern European heritage have a higher risk of primary sclerosing cholangitis.
Development
Primary sclerosing cholangitis (PSC) develops due to inflammation in the bile ducts, causing scars that lead to bile duct narrowing and eventual liver damage. There are two main types of PSC: type 1 and type 2. Type 1 PSC is linked to inflammatory bowel disease (IBD), such as ulcerative colitis or Crohn's disease. In some cases, PSC may develop before IBD, while in other cases, PSC and IBD may develop concurrently or separately over time. Type 2 PSC, on the other hand, occurs independently of IBD and is not associated with any known inflammatory bowel disease. It's important to note that the exact cause of PSC is not well understood, and both genetic and environmental factors are believed to contribute to its development.
Assessment and Diagnosis
Triage
You should see a doctor if you have severe, unexplained itching on much of your body or if you feel extremely tired all the time, no matter what you do. These symptoms may indicate primary sclerosing cholangitis, especially if you have a history of inflammatory bowel disease like ulcerative colitis or Crohn's disease. Early signs and symptoms may include fatigue, itching, yellow eyes and skin (jaundice), and abdominal pain. As the disease progresses, additional symptoms may appear, such as fever, chills, night sweats, enlarged liver, enlarged spleen, and weight loss.
Diagnosis
To diagnose primary sclerosing cholangitis, doctors typically use a combination of tests and procedures, including liver function blood tests, MRI of the bile ducts, and X-rays of the bile ducts. In some cases, a liver biopsy may be performed to determine the extent of liver damage. It is important to discuss your test results with your doctor to determine the next steps in your treatment plan.
Management and Treatment
Complications
Primary sclerosing cholangitis may lead to several complications, including:
- Liver disease and failure: Chronic inflammation of the bile ducts can cause tissue scarring (cirrhosis), liver cell death, and eventually, loss of liver function.
- Thinning bones (osteoporosis): People with primary sclerosing cholangitis may experience bone loss, which can lead to an increased risk of fractures.
- Bile duct cancer: Individuals with primary sclerosing cholangitis have an increased risk of developing cancer in the bile ducts or gallbladder.
- Colon cancer: People with primary sclerosing cholangitis associated with inflammatory bowel disease have a higher risk of colon cancer. It is essential to monitor and manage these complications to ensure the best possible outcome for individuals with primary sclerosing cholangitis.
Home Remedies
It is important to consult with a healthcare professional for appropriate medical advice and treatment options for primary sclerosing cholangitis.
Treatment
The primary treatment for primary sclerosing cholangitis is managing symptoms and monitoring liver function. When possible, procedures are done to temporarily open blocked bile ducts. However, there is no cure for the disease, and it may progress to liver failure, repeated infections, and bile duct or liver tumors. A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients.
Preparing for medical consultation
To prepare for an appointment for primary sclerosing cholangitis, you should:
- Write down your symptoms, including when they started and how they may have changed or worsened over time.
- Make a list of all your medications, vitamins, or supplements.
- Write down your key medical information, including other diagnosed conditions. Mention if you have been hospitalized in the last few months.
- Write down key personal information, including any recent changes or stressors in your life, as well as a detailed description of your typical daily diet, including whether you usually use caffeine and alcohol.
- Prepare a list of questions to ask your doctor, such as:
- What's the most likely cause of my symptoms?
- What kinds of tests do I need?
- Is my condition temporary, or will I always have it?
- What treatments are available? And what do you suggest for me?
- How can I best manage any other health conditions I have alongside primary sclerosing cholangitis?
- Are there any restrictions that I need to follow?
- Are there any brochures or other printed material that I can take with me? What websites do you recommend? By being well-prepared for your appointment, you can make the most of your time with your doctor and ensure that you receive the information and care you need for your primary sclerosing cholangitis.