AB1143 Autoimmune characteristics in a cohort of 89 patients with primary biliary cholangitis

Abstract

Background: Primary biliary cholangitis (PBC) is associated to other autoimmune diseases with an unknown prevalence. Their treatment can prevent progression to liver cirrhosis and other systemic complications Objectives: To describe the clinical and analytical autoimmune characteristics in a cohort of patients with PBC diagnosed and followed in a specific unit of Hepatic Diseases. All patients were also studied in the Autoimmune and Sistemics Diseases Unit of our hospital to check for the presence of extrahepatic autoimmune diseases. Methods: We have studied patients with PBC diagnosed in our Service since 1994 who are currently under follow-up. The diagnosis of PBC was made taking into account: the presence of colostasis enzymes with positive anti-mitochondrial antibodies (AMA) and/or compatible liver biopsy Results: We studied 89 patients with PBC. 81 of them were women (female/male ratio 10/1) with a mean age at diagnosis of 56 years (range 23-84 years). The mean follow-up was 106 months (range 9-286). IgM was elevated in 70% of the patients in whom it was found (56/80). The ANA were positive in 71% (61/86) and the AMA in 75% (67/89). 43% of patients AMA-negative PBC had other suggestive antibodies: 7 anti-centromere, 2 AMA-2 and 1 anti-sp-100. A liver biopsy was performed on 75 patients (87%), resulting in a diagnosis of 58% and useful to exclude other pathologies in the rest. In 18 patients (20%) an overlapping condition was diagnosed: PBC +Autoimmune hepatitis. In 11 patients (12%) a Reynolds syndrome was diagnosed: PBC +Scleroderma, in all of them Raynaud phenomenon was present. On another 11 of 41 (27%) Raynaud phenomenon was also present. In 17 patients (19%) there was a history or new diagnosis of autoimmune thyroiditis and in 13 patients (15%) of Sjögren syndrome. None of them was diagnosed of IgG4-related disease. Serum IgG4 was measured in 56 patients (63%) with a mean value of 36.6 mg/dL.2,8 -125 The patients with pure PBC were treated only with urodeoxycholic acid, with a complete response of 41%, a partial response of 51% and an absence of response of only 6 (7%). There was no difference regarding liver response to treatment among patients with pure PBC and patients with overlapping autoimmune hepatitis, Reynolds syndrome, Raynaud phenomenon, Sjögren syndrome nor autoimmune thyroiditis. Only 3% of patients with complete response and 12% of those who responded partially evolved to liver cirrhosis, with a similar follow-up time in both groups (105 vs 113 months). 5 of the 6 patients who did not have a biochemical response developed liver cirrhosis. 7 of the 13 patients with cirrhosis (54%) already presented clinical or histological data of cirrhosis in the initial evaluation Conclusions: PBC patients have frequently other autoimmune diseases such as Autoimmune Hepatitis, Sjögren syndrome or Scleroderma so we must actively seek the presence of these pathologies. The treatment with ursodeoxycholic seems to be useful in all patients but it is important to make an early diagnosis.