Primary Sclerosing Cholangitis

Clinic

• PSC is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder.
• Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as icteric sclera, itching, and abdominal pain.
• The bile duct scarring that occurs in PSC narrows the ducts of the biliary tree and impedes the flow of bile to the intestines.
• Eventually, it can lead to cirrhosis
• PSC increases the risk of liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma.
• The underlying cause of PSC is unknown. Genetic susceptibility, immune system dysfunction, and abnormal composition of the gut flora may play a role.
• This is further suggested by the observation that around 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.
• No effective medical treatment is known.

Signs and symptoms

Nearly half of people with PSC do not have symptoms, however signs and symptoms may include

• Severe itching
• Nonspecific fatigue
• Jaundice / Icteric sclera
• HepatoSplenomegaly 40%
• Abdominal pain 20%
• Multiple episodes of Cholangitis

• Dark urine due to excess conjugated bilirubin
• Malabsorption, esp Steatorrhea lead to decreased levels of ADEK vitamins.
• Portal hypertension

Cause

• The exact cause is unknown
• It is thought to be an autoimmune disease, thus, many experts believe it to be a complex, multifactorial.

Data have provided novel insights suggesting:

1. An important association between the intestinal microbiota and PSC and
2. A process referred to as cellular senescence and the senescence-associated secretory phenotype in the pathogenesis of PSC.

Pathophysiology

• PSC is characterized by Cholangitis with consequent Stricturing / Hardening / Sclerosis of these ducts due to scar formation, be it inside and/or outside the liver.
• The resulting scarring of the bile ducts obstructs the flow of bile, which further perpetuates bile duct and liver injury.
• Chronic impairment of bile flow due to Cholestasis causes progressive biliary fibrosis and ultimately biliary cirrhosis and liver failure.
• The primary physiological function of bile is to assist in the breakdown and absorption of fat in the intestinal tract; a relative deficiency of bile can lead to fat malabsorption and deficiencies of fat-soluble vitamins (A, D, E, K)
• Liver enlargement is seen due to portal hypertension caused by compression of portal veins by the proximate sclerosed intrahepatic bile ducts, and leads to right upper quadrant abdominal pain.

Related disease

• Cholangitis
• Cholestasis

Miasms

CMV