Sclerosing cholangitis

Disease Description (Causes and Symptoms)

• Definition and pathophysiology: A rare disease in which chronic inflammation persists in the bile ducts, causing fibrosis and thickening of the bile duct walls, resulting in irregular strictures and dilation of the bile ducts. As it progresses, it may lead to bile stasis, portal hypertension, and cirrhosis. It is classified into primary (idiopathic) and secondary forms caused by other diseases.
• Causes: The exact cause is unknown, but autoimmune mechanisms, toxins or bacterial infections, and pancreatic or retroperitoneal fibrosis may be involved. The primary form is strongly associated with inflammatory bowel diseases such as ulcerative colitis. It is approximately twice as common in men.
• Main symptoms: Fatigue, weight loss, loss of appetite, jaundice, itchy skin (pruritus), right upper quadrant pain, and fever and chills (when acute cholangitis occurs). In some cases, it is discovered incidentally during testing despite the absence of symptoms.
• Risk of complications: Cirrhosis, liver failure, cholangiocarcinoma (high incidence of bile duct cancer), intrabiliary stones and recurrent cholangitis (risk of sepsis), portal hypertension and related bleeding, etc.
• Diagnosis (summary): Blood tests may show abnormal liver function, particularly elevated ALP and GGT. On imaging, irregular strictures and dilation of the bile ducts are characteristic findings on MRCP (noninvasive) and ERCP (diagnostic and therapeutic). If necessary, liver biopsy may be used to support the diagnosis.

Treatment Methods

• Medication
  • Ursodeoxycholic acid (UDCA): May help improve liver function test results, but its clearly established effects on preventing disease progression and complications are limited.
  • Antibiotics for acute cholangitis (selected according to blood and culture results).
  • Treatment of itching: Symptom-relieving medications such as cholestyramine and ripaperidone; phototherapy or experimental medications may be considered in severe cases.
  • Fat-soluble vitamins (A, D, E, and K) may be administered when supplementation is needed.
• Endoscopic and interventional procedures
  • First-line interventions for strictures, stones, and drainage problems include balloon dilation, stent placement, and biliary drainage through ERCP.
  • Alternative drainage methods such as percutaneous transhepatic biliary drainage (PTBD).
  • Caution is required regarding procedural complications such as pancreatic abscess, bleeding, and infection.
• Surgery and liver transplantation
  • Liver transplantation is the definitive treatment: The reported 5-year survival rate after liver transplantation is approximately 85%, with relatively favorable outcomes reported in the literature.
  • Reports indicate a concomitant cholangiocarcinoma rate of approximately 10–36% at the time of transplantation. When cholangiocarcinoma is present, the prognosis is very poor (a 1-year survival rate of approximately 30% has been reported), making early evaluation and determining the timing of transplantation important.
  • Regular examinations are necessary because immunosuppression after transplantation increases the risk of colorectal cancer, particularly in patients with a history of ulcerative colitis.
• Lifestyle management and preventive measures
  • Avoid alcohol and hepatotoxic medications, monitor liver function regularly, and maintain proper nutrition, including supplementation with fat-soluble vitamins.
  • Because of the association with ulcerative colitis, regular surveillance with colonoscopy is recommended; discuss the detailed examination schedule with the attending physician.
  • Vaccination, including hepatitis vaccines, and minimizing the risk of infection.
• Precautions
  • Regular monitoring is necessary because the disease may progress even without symptoms.
  • Follow-up is essential to monitor procedure- and surgery-related complications during and after treatment, as well as long-term cancer risks such as cholangiocarcinoma and colorectal cancer.

Checklist Before an Outpatient Visit (Including Example Questions)

• Items and records to bring
  • A list of currently used medications, including prescription drugs, over-the-counter medications, and herbal medicines
  • Previous test results, including blood tests, ultrasound, MRCP/ERCP/CT imaging files, and copies of liver biopsy results
  • A recent symptom diary, including the timing and frequency of jaundice, itching, fever, changes in weight, abdominal pain, and other symptoms
  • Medical history: Whether inflammatory bowel disease, particularly ulcerative colitis, is present; history of liver transplantation; surgical history; and allergies
  • Lifestyle information: Amount of alcohol consumption, smoking, and occupational exposure to toxins
• Key information to tell the doctor during the visit (example questions)
  • "When did the recent jaundice and itching begin, and are there any factors that make them worse?"
  • "Have you recently had a fever or chills (suggestive of cholangitis)?"
  • "Do you have a history of being diagnosed with ulcerative colitis? When was your most recent colonoscopy?"
  • "Are you currently taking any supplements or medications?"

Examples of General Questions to Ask the Doctor

• What effects and side effects can be expected from taking this medication, such as ursodeoxycholic acid?
• Based on my current test results, how advanced is the disease, including cirrhosis and reduced liver function?
• What are the criteria for needing ERCP or a stent procedure? What are the risks of the procedure?
• When is liver transplantation recommended, and what should be considered before and after transplantation?
• What regular tests for cholangiocarcinoma and colorectal cancer should I undergo, such as hepatobiliary imaging, tumor markers, and colonoscopy, and how often?
• If I am planning a pregnancy, how could this affect disease management and treatment choices?

Examples of Disease-Specific Questions

• Even if I do not have a history of ulcerative colitis, should I undergo regular colonoscopy? (Including the examination interval and reasons)
• Which test is more appropriate for me, MRCP or ERCP, and what are the advantages, disadvantages, and risks of each?