General

Primary Biliary Cirrhosis (PBC)

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10 Sep 2016 - General
 

Primary biliary cirrhosis (PBC) mainly affect women in their sixth decade of life. It is the autoimmue disorder of liver, with unkown underlying specific cause of it. The serum autoantibodies related to primary biliary cirrhoiss are serum anti mitocrondrial antibody (anti AMA), anti AMA-M2. Serum IgM level will be elevated in around 90% of patients with primary biliary cirrhoiss. The are a few diseases that can be associated with primary biliary cirrhosis, like sjogren syndrome, autoimmune thyroiditis, scleroderma/CREST, rheumatoid arthritis, dermatomyositis, mixed connective tissue disease, systemic lupus erythematosus, renal tubular acidosis, and idiopathic fibrosis. Sometime, liver biopsy may be needed in the diagnosis of this condition.

The diagnosis of PBC can be established when two of the following three criteria are met:
● Biochemical evidence of cholestasis based mainly on alkaline phosphatase elevation.
● Presence of AMA.
● Histologic evidence of nonsuppurative destructive cholangitis and destruction of interlobular bile ducts.
Bile acid sequestrants should be used as initial therapy for patients with PBC who have pruritus. The following agents can be used for pruritus refractory to bile acid sequestrants:...
 (Total 97 words)
Most patients with primary biliary cirrhosis experience a progressive clinical course, that may result in cirrhosis eventually. Some asymptomatic patients will have a longer median survival than the symptomatic cohort of patients. But, comparing to the sex-matched and age-matched general population, the asymptomatic cohort of patient with primary biliary cirrhosis do have a reduced median survival. Estimate overall median survival without a liver transplantation will be ranging between 10 to 12 ...
 (Total 113 words)
Primary billiary cirrhosis or known as primary billiary cholangitis is an auto immune disease which can affect liver architecture leading end stage liver disease. The usual symptoms are fatigue, right upper quadrant discomfort and pruritus. Physical examination is a must in these patient and reveals hepatomegaly, hyperpigmentation, splenomegaly or jaundice. In advanced disease patients can present with spider naevi, ascitis, palmar erythema, proximal muscle wasting and peripheral edema. Investi...
 (Total 102 words)
Primary biliary cirrhosis is a chronic disease of the liver that is autoimmune in nature. It may lead to progressive cholestasis or even end-stage liver disease. It is more common in women especially between the fourth and sixth decade of life. PBC develops when bile ducts of the liver are destroyed and bile is not supplied properly to the liver as bile juice is very necessary for digesting food and removing worn out RBCs, cholesterol, and toxins. This is the reason PBC is so harmful and conside...
 (Total 107 words)