Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis is a progressive disorder characterized by inflammation, fibrosis and stricturing of medium and large-sized ducts in the intrahepatic and or extrahepatic biliary tree. It ultimately causes biliary cirrhosis, portal hypertension and hepatic failure. Cholangiocarcinoma develops in 10 to 30% of patients in the course of the disease.

Associated conditions;

• Inflammatory bowel disease
• Retroperitoneal fibrosis
• Chronic pancreatitis
• Riedel’s thyroiditis
• Retro orbital tumours
• Angioimmunoblastic lymphoma

Secondary sclerosing cholangitis occurs when duct fibrosis can be attributed to an apparent predisposing factor. The causes are;

• Bile duct stones causing cholangitis
• Previous billed duct surgery with structures and cholangitis
• Insertion of formalin into hepatic hydatid cysts
• Parasitic infections
• Autoimmune pancreatitis
• AIDS, etc

Pathophysiology

• It has a strong association with ulcerative colitis. Patients who have both are at increased risk of colorectal neoplasia.
• It is currently believed to be an autoimmune disorder triggered in genetically susceptible individuals by toxic or infectious agents.
• Perinuclear antineutrophil cytoplasmic antibodies(ANCA) are present in 60 to 80% of patients.

Clinical presentation

• Maybe asymptomatic and be discovered incidentally by the persistently elevated ALP.
• Commonly present with right upper quadrant abdominal pain, fatigue, intermittent jaundice, weight loss and pruritus

Physical examination

• Most have a normal physical examination
• Findings- jaundice, hepatomegaly, splenomegaly, excoriations

Investigations

1. LFT- cholestatic picture. Low albumin and clotting abnormalities are characteristics of a late stage of the disease.
2. Serum perinuclear ANCA are present
3. Hypergammaglobulinemia and raised serum IgM
4. MRCP – the critical investigation. Shows multiple strictures and dilatation
5. ERCP- reserved for intervention
6. Liver biopsy- onion skin fibrosis with portal oedema and bile ductular proliferation are present. Later, fibrosis occurs. Obliterative cholangitis leads to the ‘vanishing bile duct syndrome.’

Management

Goals;

• Retardation and reversal of the disease process
• Control of the disease and its complications

1. Drug therapy;

• Ursodeoxycholic acid( UDCA)- protects cholangiocytes from hydrophilic bile acids, stimulates hepatobiliary secretion, protects hepatocytes against bile acid-induced apoptosis and induces antioxidants. It may have benefits in reducing colon carcinoma risk.
• Broad-spectrum antibiotics, e.g. ciprofloxacin for acute attacks of cholangitis, have no role in preventing attacks.
• Fat-soluble vitamin replacement in jaundiced patients
• Treatment of osteoporosis when it occurs
• Cancer screening

1. Endoscopic therapy for strictures
2. Surgery;

• Biliary reconstruction
• Liver transplantation- although the disease may recur in the graft
• Proctocolectomy

Prognosis

• There’s no cure, and the course is variable
• Median survival of 12 years
• Most die from liver failure, 30% die from cholangiocarcinoma and the rest from colonic cancer.