ABSTRACT
Recently it has been described in the literature a subpopulation of PBC patients with negative AMA that might represent patients with ANA positive autoimmune cholangitis. We review 25 cases of PBC (23 females) with AMA negative. Our aim was to determine the frequency of ANA(+) in this group. We studied serum transaminases, total bilirubin, AMA and ANA by indirect immunofluorescence considering positive dilutions of 1:40. All patients had elevated alkaline phosphatase (641 +/- 389 U/l). Total bilirubin was below 2.5 in 59%. Thirteen patients had esophageal varices. The histologic stage was I-II in eleven and III-IV in 14 cases. Fourteen patients (56%) has ANA(+) (dilution 1:40), 44% had lower dilutions. We confirm the presence of a subpopulation of PBC AMA negative, ANA positive patients and the observations of different and diverse immune alterations in PBC patients.