DOES ANTIMITOCHONDRIAL ANTIBODY STATUS AFFECT RESPONSE TO TREATMENT IN PATIENTS WITH PRIMARY BILIARY-CIRRHOSIS - OUTCOMES OF URSODEOXYCHOLIC ACID THERAPY AND LIVER-TRANSPLANTATION

Approximately 5% to 10% of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodie s (AMA). Most of these patients have other autoantibodies, a syndrome recently named ''autoimmune cholangitis.'' We report our experience in patients with AMA-negative PBC treated with ursodeoxycholic acid (UDC A) and/or liver transplantation (OLT), The study of response to UDCA w as performed as follows. While recruiting patients for a previously re ported multicenter trial, we identified 8 patients with AMA-negative P BC. The patients were given UDCA and followed up at regular intervals, The characteristics of AMA-negative patients at presentation were sim ilar to those of AMA-positive patients with PBC. The clinical outcomes and sequential liver biochemistries of UDCA treatment were also compa rable with those of AMA-positive patients, The study of outcome of OLT was performed as follows. We identified OLT recipients at the Mayo Cl inic who had clinical, radiological, and histological features compati ble with PBC. Their pretransplant AMA status was determined, and each AMA-negative patient was paired with 2 AMA-positive patients, Of 85 OL T recipients with a diagnosis of PBC, 6 (7.1%) were AMA negative, incl uding 1 who had undergone UDCA therapy. After a median of 36 months of follow-up, graft and patient survival rates and subsequent histologic al changes (disease recurrence and steroid-resistant or late rejection s) were comparable in AMA-negative and -positive PBC patients. In summ ary, in our experience of 13 AMA-negative PBC patients (including 9 wh o met the criteria for a diagnosis of autoimmune cholangitis), treatme nt with UDCA or OLT resulted in similar outcomes to those found in AMA -positive patients. We conclude that AMA status does not affect the re sponse in PBC patients to treatment with UDCA or OLT.