Primary Sclerosing Cholangitis | Symptoms, Causes and Treatment
Primary Sclerosing Cholangitis
- Primary sclerosing cholangitis (PSC) is an uncommon disease consisting of characteristic obliterative fibrosis of the whole or part of the biliary tree.
- It is benign and non-bacterial in nature.
- Etiology is unknown.
- Ulcerative colitis is found in 3/4th of cases and is postulated as a factor responsible for PSC.
- Possibility of an autoimmune basis is suggested in 60% cases.
- Pathogenesis
- Inflammation and fibrosis usually affect sub-mucosa and outer fat layer of bile duct.
- Process involves entire biliary tree.
- Gall-bladder may also be involved.
Symptoms of Primary Sclerosing Cholangitis
- Jaundice id detected in many cases at an asymptomatic stage.
- Initially patient complaints of jaundice and itching sensation.
- There may be upper abdominal pain, weight loss and occasionally fever.
- Liver enlargement is seen in some cases.
- In later stage splenomegaly and ascites may be found with development of secondary cirrhosis.
Diagnosis of Primary Sclerosing Cholangitis
- Generalized beading and stenosis of biliary tree.
- Absence of gallstones.
- Exclusion of bile duct cancer.
- Laboratory investigations reveal cholestasis and alkaline phosphate is raised.
- There are no diagnostic immune markers.
- ERCP is used for confirming the diagnosis.
- PSC is closely associated with ulcerative colitis however, symptomatic colitis is mild.
Primary Sclerosing Cholangitis Treatment
- Fat soluble vitamins and other supplement are used.
- Specific therapy with corticosteroids or other immuno-suppressants have been tried with variable and unsatisfactory results.
- Endoscopic treatment allows dilatation of major strictures and removal of biliary sludge and debris. They have an impact on morbidity but effect on survival is still unknown.
- Liver transplant is the only available therapeutic modality with a 3yrs survival rate of 85%. However, recurrences and even cholangio-carcinoma can develop in post transplant period.
