[Primary sclerosing cholangitis (PSC)--humoral immune phenomena, pathogenetic aspects and therapeutic possibilities]

There are about 80% antibodies in PSC against cytoplasmatic antigens of neutrophilic granulocytes of the perinuclear type (pANCA), inconstantly there are antinuclear antibodies (ANA) too, but no antimitochondrial antibodies. The frequent association of PSC with colitis ulcerosa suggests an enterobacterial aetiopathogenesis. PSC sera show clear bands at 60-90 kD and at about 10 kD in the immunoblot with enterobacterial proteins as antigens. Antibodies against enterobacterial lipopolysaccharides and lipid A are to be found in patients with PSC corresponding to the normal collective. After long-term immunization with enterobacterial antigens PSC-like changes with circulating ANA can be induced in mice and rabbits. PSC, comparable to primary biliary cirrhosis, also reacts to treatment with ursodesoxycholic acid but it scarcely reacts to immunosuppressive therapy. At the final stage of the disease liver transplantation is indicated. In our clinic up to now 16 patients with PSC have undergone a transplantation with a one-year-survival rate of 88%. Confirmed re-manifestations of PSC in the transplant have not been diagnosed up to now.