Primary sclerosing cholangitis (PSC) is a progressive scarring of the liver’s bile ducts. According to current estimates, between 5 and 16 in every 100,000 people have PSC.
The symptoms of PSC depend on how advanced this progressive condition is. Over time, PSC causes increasing tissue damage and can lead to liver failure, among other issues.
Treatments for this condition focus on slowing the progression of the disease and managing associated complications. Fortunately, there are several options available for PSC.
When assessing cases of PSC, healthcare providers break the condition into three primary subtypes: classic, small duct, and autoimmune hepatitis-associated PSC.
Classic
The liver has both small and large bile ducts. Classic PSC causes scarring of both types of ducts. This type is the most common form, accounting for 90% of cases.
Small Duct
As the name suggests, small duct PSC only affects small bile ducts. It accounts for 5% of all cases. This type tends to have a better prognosis and is the least severe of the three. It can, however, progress to the classic type.
Autoimmune Hepatitis-Associated
Autoimmune hepatitis occurs when the immune system mistakenly attacks the liver. This sometimes leads to a form of PSC that can affect both large and small bile ducts. This type is generally less severe than the classic type, but it has a worse prognosis when compared to the small duct PSC.
The symptoms of PSC depend on how far the condition has advanced. The condition may not initially cause symptoms. Upon diagnosis, about half of people do not yet have any signs. As PSC progresses, the symptoms worsen and evolve, especially if there’s a bile duct infection.
Common Symptoms
The initial symptoms of PSC include those in the abdomen and throughout the body. You might notice the following:
- Abdominal pain
- Pruritis (itchiness)
- Diarrhea, or loose and watery stools
- Jaundice, a yellowing of the skin and whites of the eyes
- Fatigue or muscular weakness
- Fever
Bile Duct Infection Symptoms
As PSC progresses, it can cause infection and inflammation of the bile ducts. This interrupts the flow of bile, causing symptoms like a new or worsening case of jaundice, chills, and sharp pains in the upper-right quadrant (quarter) of the abdomen.
Advanced Symptoms
When PSC gets severe, it causes cirrhosis (liver scarring). Symptoms at this stage vary and include:
- Ascites, or swelling in the abdomen
- Hepatic encephalopathy, which causes confusion or difficulty concentrating or thinking
- Bleeding in the intestinal tract, stomach, or esophagus (tube connecting the throat to the stomach)
- Edema, or swelling in the limbs
- Jaundice
- Weight loss
PSC is a disease of the bile ducts, the “gates” through which bile or digestive fluid exits. It arises when these ducts become inflamed and cause fibrosis (develop into scar tissue). Bile builds up in the liver, leading to further damage and, eventually, organ failure.
Researchers are unsure what causes PSC, but they think it arises from a combination of genetic and environmental factors. They believe PSC is an autoimmune disorder in which the immune system mistakenly attacks bile duct cells. People with human leukocyte antigen (HLA) gene mutations have certain environmental triggers that cause this autoimmune response.
Risk Factors
Several health factors increase your chances of developing PSC. These include:
- Being assigned male at birth, among whom PSC is twice as common
- Having inflammatory bowel disease (IBD), a set of conditions that includes ulcerative colitis and Crohn’s disease
- Being in your 30s or 40s
- Having a family member with PSC
- Having an autoimmune disorder, such as type 1 diabetes or thyroid disease
Part of the healthcare provider’s job when diagnosing PSC is to rule out other potential causes of symptoms. These include different types of cholangitis (inflammation of the bile ducts), human immunodeficiency virus (HIV), and primary biliary cirrhosis (a chronic liver disease).
In addition to taking your medical history and asking about any family history of the condition, the healthcare provider may perform the following tests:
- Liver function tests: These tests identify certain proteins and enzymes in your blood, such as albumin and bilirubin, that can indicate signs of infection or damage.
- Magnetic resonance cholangiopancreatography (MRCP): This form of MRI allows healthcare providers to examine the affected bile ducts or liver tissue.
- Endoscopic retrograde cholangiopancreatography (ERCP): This type of imaging combines X-ray and endoscopic methods (using a tube with a camera) to assess bile ducts.
- Percutaneous transhepatic cholangiography (PTC): This form of X-ray relies on an injected contrast dye to help providers assess affected areas.
- Other imaging: Ultrasounds and computerized tomography (CT) are other imaging methods used to assess bile ducts.
- Liver biopsy: This procedure involves testing a sample of liver tissue for signs of damage or cancer. It is used to rule out other potential causes of your symptoms but is otherwise not needed for diagnosis.
Because PSC is a chronic and progressive condition, there is no outright cure for it. That said, treatments work to reopen or repair damaged bile ducts and manage symptoms, with surgeries becoming necessary for complications and advanced cases.
Minimally Invasive Procedures
Several minimally invasive techniques focus on repairing damaged bile ducts. Using ERCP imaging to guide their work, surgeons open blocked or narrowed bile ducts. They place stents—small mesh cages or tubes—or inflate balloons to hold them open.
Medications
Over-the-counter (OTC) and prescribed medications can treat the itching caused by PSC. These include:
- Prevalite (cholestyramine), a cholesterol medication
- Rifadin (rifampin), an antibiotic often indicated for tuberculosis (TB)
- Vivitrol (naltrexone), a medication for narcotic withdrawal
- Certain antibiotics (for bile duct infection)
- Blood pressure medications, such as beta-blockers or calcium channel blockers
Ursodiol, a medication that dissolves gallstones, may be effective in treating this condition. However, medical experts do not agree on the off-label use of this drug.
Surgery
Several surgeries are available for PSC and liver complications. These include:
- Choledochoduodenostomy: The surgeon connects the common bile duct to the duodenum, the upper part of the small intestine running from the stomach. This allows bile to escape the liver.
- Choledochojejunostomy: This surgery involves connecting the common bile duct to the jejunum, the middle portion of the small intestine.
- Liver transplantation: After a certain point, the liver damage leads to organ failure; these cases call for liver transplant surgery.
While there’s no way to prevent PSC, lifestyle changes may help boost your liver’s health. This involves eating a healthy, well-balanced diet and limiting or avoiding alcohol—especially if you have liver cirrhosis.
Your healthcare provider may also recommend vitamin supplementation. Vitamin D and calcium supplements help prevent osteoporosis, a complication that causes thinning or weakening bones. Vitamin A, D, and E supplements may help PSC-associated vitamin deficiencies.
Over time, PSC can cause serious complications and raise the risk of certain conditions. These include:
- Inflammatory bowel disease (IBD): About 70% of those with PSC also have IBD. IBD is a set of conditions that impact the intestines, including ulcerative colitis and Crohn’s disease.
- Vitamin deficiency: Because vitamins are absorbed in the liver, PSC can cause persistent deficiencies in vitamins A, D, and E.
- Osteoporosis: Osteoporosis, a thinning and weakening of the bones, is a complication of PSC that occurs in about 15% of cases.
- Gallbladder diseases: Gallstones, polyps (growths), and gallbladder cancer are also associated with PSC; up to 1 in 4 patients with PSC develop gallstones.
- Cancer: Liver cirrhosis due to PSC raises the risk of several types of cancer, including cholangiocarcinoma (a cancer of the bile ducts) and colon cancer. About 10-20% of patients develop the former.
- Biliary stricture: This complication is a permanent narrowing and blockage of the bile ducts.
- Cholangitis: This condition involves inflammation and swelling of the bile ducts and causes fever, chills, abdominal pain, and jaundice.
PSC is a chronic condition that causes slow, progressive damage to the liver. Patients survive an average of 10-20 years after diagnosis. That said, small duct PSC does not always lead to cancer or liver failure and has a better prognosis.
About 40% of those diagnosed with this condition require a liver transplant, and the surgery has a high success rate. According to research, 85% of patients survived one year after treatment, and 72% were alive at five years. However, PSC can return to transplanted livers.
Living with a chronic disease like PSC can take a heavy toll. Some methods may help you cope, including:
