Abstract
We present the case of AMA-negative PBC A 21 yearold woman presented with a 6 year history of disease. The first manifestation was dyspeptic syndrome with mild jaundice and was diagnosed with hepatopathy of unknown origin. In September 2016 her condition worsened. Investigations revealed elevated ALP, AST, ALT, GGT, total and direct bilirubin, abdominal ultrasound revealed hepatosplenomegaly, mild ascites. An upper GI tract endoscopy revealed grade I esophageal varices. Other causes of hepatitis were repeatedly excluded. ANA, AMA and ASMA by indirect immunofluorescence were negative, AMA was investigated by immunoblotting method and result was negative. Liver biopsy revealed multiple macroscopic nodules separated by broad fibrous septae with mixed inflammatory cell infiltrates and bile duct injury suggestive of biliary cirrhosis. Thus, here is the case report of the patient with AMA negative Primary Biliary Cirrhosis, where diagnosis was confirmed by liver biopsy. Condition of the patient became stable after starting treatment with UDCA (1000 mg) and currently patient is under observation.
References
2. Gong Y, Huang Z, Christensen E, Gluud C (2007) Ursodeoxycholic acid for patients with primary biliary cirrhosis; an updated systematic reviw andmeta-analisis of randomized clinicaltrials. Am J Gastroenterol. 102: 1799-1807.
3. Keith DL, Eric GM, Raoul P (2009) Primary Biliary Cirrhosis. Hepatology 50: 291-308.
4. Leuschner U (2003)Primary biliary cirrhosis—presentation and diagnosis.Clin Liver Dis7:741-758.
5. Michieletti P, Wanless I, Kats A, Scheuer P (1994) Antimitochondrial Antibody-Negative Primary Biliary Cirrhosisa distinct syndrome of autoimmune cholangitis. Gut 35: 260-265.
6. Juliusson G, Imam M, Bjomsson ES, Talwalkar JA. Long-term outcomes in antimitochondrial antibody negative primary biliary cirrhosis. Scand J Gastroenterol 51: 745-752.