Abstract
A male case of primary biliary cirrhosis (PBC) with lupoid hepatitis is reported. A 53-year-old man was admitted to our hospital because of liver dysfunction. Laboratory data showed high levels of serum Al-p (22 KAU), LAP and y-GTP (387mU/ml), and amount of serum IgG elevated extremely up to 3566mg/dl. Anti mitochondrial antibody (AMA), and smooth muscle antibody (ASMA), anti nuclear antibody (ANA), and DNA and anti SS-B were positive. Using extracts of bovine heart mitochondria as a source of antigen, patients serum such as other PBC sera showed two precipitating lines by double immunodiffusion test. However, the subtype of AMA was anti-M2, not anti-M4.
Histological examination of surgical biopsy showed typical chronic non-suppurative destructive cholangitis (CNSDC), enlarged portal tracts with heavy imflammatory cells mainly composed of lymphocytes and plasma cells, and piecemeal necrosis characterized by chronic active hepatits (CAH). The levels of ANA, anti-DNA and transaminase were well responded to steroid therapy.
Therefore, this case was diagnosed as overlapping case of asymptomatic PBC and lupoid hepatitis although anti-M4 was not detected. It would be difficult to diagnosis CAH-PBC mixed form by reason of positive or negative anti-M4.
