Concomitant Case of Primary Biliary Cirrhosis and Autoimmune Hemolityc Anemia Responding to Corticosteroid and Ursodeoxycholic Acid in Young Woman
Abstract: Primary biliary
cirrhosis (PBC) is an autoimmune liver disease of unknown etiology and is
characterized by chronic progressive cholestasis with destruction of the small
intrahepatic bile ducts and associated most commonly with antimitochondrial
antibodies. PBC is most common in women and is often associated with other
autoimmune disease such as autoimmune hemolityc anemia (AIHA), rheumatoid
arthritis, thyroiditis, and systemic lupus eritomatosus.
We report one case, a 20 years old woman with AIHA have been treated by
corticosteroid since last year and she came to the outpatient department (OPD)
with fatique and jaundice. The result of laboratory were haemoglobin 8.7 mg/dL,
white blood cell 8700 mg/dL, coomb test +2, total bilirubin 33.2 mg/dL, direct
bilirubin 29.3 mg/dL, γGT: 297 mg/dL and alkalyphospatase: 158 mg/dL. The
result of Abdominal CT scan showed the size of liver and spleen increased and
normal common bile duct (CBD). The result of ANA test, anti-nuclear (ANA) and
antimitochondrial M2 (AMA M2) antibodies were positive. From the physical
examination, laboratory and CT scan Abdomen; the diagnose of this patient was
AIHA with PBC.
After treatment with corticosteroid (prednison 1mg/kg/day) and
ursodeoxicholic acid (UDCA) for several weeks, the clinical manifestation of
PBC such as jaundice getting better (the laboratory result: total bilirubin 2.7
mg/dL, direct bilirubin 1.5 mg/dL, gamma GT 80 mg/dL).
Keywords: primary biliary
cirrhotic; autoimmune hemolityc anemia; corticosteroid; ursodeoxicholic acid
Author: Susanto H Kusuma,
Fardah Akil, Amelia Rifai, Rini R Bachtiar, Numan AS Daud, AM Luthfi Parewangi
Journal Code: jpkedokterangg160425