Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are both chronic bile duct diseases. They are rare diseases of autoimmune origin that share many of the same symptoms, but there are many ways in which they differ.

What Are PBC And PSC?

With PBC, the bile ducts inside the liver become inflamed and begin to break down. As the disease progresses, it becomes harder for bile to flow through the ducts. This causes bile to back up into the liver where it causes damage that leads to scarring known as cirrhosis. While the damage to the liver occurs in the same way with PSC as in PBC, the bile ducts inside and outside of the liver are affected in PSC. In both diseases, the damage caused to the liver often leads to liver failure.

How Are PBC And PSC Different?

Aside from the bile ducts involved, these two diseases differ in who they affect, how they are diagnosed and how they are treated.

  • Who They Affect: Although they can both happen to anyone at any age, PBC occurs mostly in women while PSC is more common in men.
  • Disease Comorbidity: The majority of patients diagnosed with PSC (up to 80%) also have inflammatory bowel disease (IBD). In PBC, the incidence of IBD is much lower at about 1%.
  • How They Are Diagnosed: PBC is usually diagnosed via a blood test. PSC diagnosis requires an imaging test. A liver biopsy may be conducted to assess liver damage with either disease.
  • Medical Treatment: Although there is no cure, medication can slow the progression of PBC and improve its symptoms. There is no proven, effective treatment for PSC.
  • Endoscopic And Surgical Treatment: Endoscopic therapy and surgical procedures can improve bile flow in patients with PSC. In both diseases, liver transplant is the best treatment option if the disease progresses to end-stage liver disease.
  • Cancer Risk: There is an increased overall cancer risk with both diseases. PBC has a greater associated risk for liver cancer. PSC has a higher risk for bile duct, liver, gall bladder and colon cancers.

Symptoms And Causes

While both diseases often have no symptoms in the early stages, the most common first symptoms are fatigue and itching. As the disease progresses, patients can experience the following symptoms:

  • Abdominal pain (upper right)
  • Darkening of the skin
  • Diarrhea
  • Dry eyes and mouth
  • Fatty lesions on the skin called xanthomas
  • Fever
  • Jaundice (yellowing of the skin and/or eyes)
  • Joint pain
  • Swelling in the abdomen and/or legs
  • Unexplained weight loss

Like all autoimmune diseases, the exact cause of PBC and PSC is unknown. What we do know is that genetics likely play a role, along with environmental factors. PSC is also highly associated with IBD, especially ulcerative colitis.

Outlook For Patients With PBC Or PSC

In most cases, both diseases progress slowly. Early diagnosis and treatment may delay or even prevent liver failure and other late-stage complications. The average life expectancy from diagnosis is similar for both diseases: between 10 and 20 years. This can be longer for patients who are asymptomatic when diagnosed or who receive a liver transplant if liver failure develops.

If you have any symptoms of a liver issue, routine checkups and prompt evaluation are paramount to preventing or managing liver disease. You should see your doctor if you have any signs or symptoms of liver disease, such as:

  • Abdominal pain or swelling
  • Dark urine
  • Fatigue
  • Itchy skin
  • Jaundice
  • Light-colored stool
  • Nausea and/or vomiting
  • Swelling in the ankle or leg

The team at GastroIntestinal Specialists, A.M.C. treats multiple conditions and diseases of the gastrointestinal (GI) tract. Our board-certified physicians have more than 150 years of combined experience in providing quality care you can trust. To schedule an appointment, call (318) 631-9121 or visit gis.md.