Primary biliary cirrhosis in brothers

Summary This is the first report of two brothers who demonstrated the classical clinical course, histology, biochemistry and auto-antibodies of primary biliary cirrhosis. Both also exhibited an associated keratoconjunctivitis sicca and, in one, renal tubular acidosis resulted in severe systemic acidosis after lactulose therapy and with a subsequent intraperitoneal variceal rupture. Screening of the relatives recalled a high incidence of ‘auto-immune’ disease and auto-antibodies.