Primary Biliary Cirrhosis: -Idiopathic chronic progressive inflammatory autoimmune liver disease, - Immune-mediated destruction of small and medium-sized intra-hepatic ducts -Diagnosis is made when 2 of 3 criteria are fulfilled : 1. Biochemical evidence of cholestatic liver disease for at least 6 month’s duration 2. Anti-mitochondrial antibody (AMA) positivity 3. Histologic features of PBC on liver biopsy,i.e, - nonsuppurative cholangitis -destruction of small or medium-sized bile ducts Incidence -10:1 female to male ratio -usually affects in fifth decade of life Symptoms-most frequent - Fatigue and pruritus -Pruritis-specific for PBC ,can be localised or diffuse,worse at night -Fatigue Biochem-elevated GGT, ALP,can have elevated transaminases. Antibodies- PDC-E2 specific AMA(antimicrobial antibody)detectable in approximately 90% Investigation-If there is biochemical evidence of cholestasis- Do USG of liver and biliary tree. If the biliary system is normal on ultrasound + AMA positive, no further radiographic testing is necessary. -If the diagnosis - uncertain with noninvasive testing, cholangiography maybe be necessary . -Liver biopsy - for the diagnosis of AMA-negative PBC -exclude other concomitant disease such as autoimmune hepatitis (AIH) and NASH. - prognosticate Complications- Cirrhosis - increased risk for hepatocellular carcinoma -screen with cross-sectional imaging every 6-12 months, with or without alpha fetoprotein -Osteoporosis-most common bone disorder seen in PBC . - Sicca Syndrome- dry eyes and mouth(also seen in PBC) Treatment- First line therapy -UDCA (ursodeoxycholic acid) -dose of 13-15mg/kg. -Calcium,Vit D,bisphosphonates for osteoporosis. -Consider liver transplantation. Source-ncbi.nlm.nih.gov/pmc/articles
Posted on: Sat, 08 Jun 2013 10:40:31 +0000
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