Primary Sclerosing Cholangitis (PSC)

Overview:

Primary Sclerosing Cholangitis (PSC) is a chronic liver disease characterized by

inflammation and scarring (fibrosis) of the bile ducts inside and outside the liver. This

condition leads to the obstruction of bile flow, causing liver damage over time. PSC is

considered an autoimmune disease, although its exact cause is not fully understood.

Causes:

The precise cause of PSC is unknown, but it is believed to involve an autoimmune

response, where the immune system mistakenly attacks the bile ducts. Genetic factors

may also play a role in predisposing individuals to PSC. The condition is often

associated with other autoimmune diseases, such as inflammatory bowel disease

(IBD).

Symptoms:

PSC can be asymptomatic in its early stages, but as the disease progresses, symptoms

may develop. Common symptoms include:

● Fatigue: Persistent tiredness and weakness.

● Itching (Pruritus): Itchy skin, often severe, is a common symptom due to the

accumulation of bile salts.

● Abdominal Pain: Discomfort or pain in the upper right abdomen.

● Jaundice: Yellowing of the skin and eyes due to impaired bile flow.

● Weight Loss: Unintentional weight loss may occur in advanced stages.

● Complications: PSC is associated with complications such as cirrhosis, liver

failure, and an increased risk of bile duct and liver cancer.

Treatment:

The treatment of PSC focuses on managing symptoms, slowing disease progression,

and addressing complications. Common approaches include:

● Ursodeoxycholic Acid (UDCA): This medication is commonly prescribed to

improve bile flow and may have a beneficial effect in some individuals.

● Symptomatic Relief: Medications may be prescribed to alleviate symptoms such

as itching.

● Endoscopic Procedures: Balloon dilation or stent placement may be performed to

open narrowed bile ducts.

● Liver Transplant: In advanced stages with cirrhosis and liver failure, a liver

transplant may be considered. PSC has a high likelihood of recurrence in the

transplanted liver.

● Monitoring and Surveillance: Regular monitoring with imaging studies and blood

tests to detect and manage complications, including the risk of cancer.

PSC is a complex and challenging condition to manage. Individuals with PSC often

require ongoing care and monitoring by a multidisciplinary healthcare team, including

hepatologists, gastroenterologists, and transplant specialists. Early diagnosis and

intervention are crucial for optimizing outcomes and improving the quality of life for

those affected by PSC.