NEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AFTER LIVER-TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS

The immunopathogenic importance of neutrophil cytoplasmic autoantibodi es in ulcerative colitis and primary sclerosing cholangitis is unknown . These autoantibodies were investigated before and after liver transp lantation in 9 patients with primary sclerosing cholangitis. Sera from 10 patients transplanted for metabolic disorders or hemangioma served as controls. Before liver transplantation neutrophil cytoplasmic auto antibodies, producing a perinuclear pattern by indirect immunofluoresc ence on ethanol fixed neutrophils, were present in all patients with p rimary sclerosing cholangitis. A decline in titer was noted in the fir st months after liver transplantation. During long-term follow up, the autoantibodies remained present and most often the titer did not diff er from before transplantation. They were not directed against protein ase 3, myeloperoxidase, elastase or lactoferrin. All but one of the co ntrol patients were negative for the autoantibody. No relation was see n, before or after transplantation, with ulcerative colitis or proctoc olectomy. There was no recurrence of primary sclerosing cholangitis in any of the patients as judged by liver histology. We conclude that ne utrophil cytoplasmic autoantibodies remain present after liver transpl antation for primary sclerosing cholangitis and that its synthesis is not related to the presence of the diseased organ(s). The primary dise ase process in primary sclerosing cholangitis and ulcerative colitis m ay well be a disturbance of the immune system.