Low incidence of primary biliary cirrhosis (PBC) in the first‐degree relatives of PBC probands after 8 years of follow‐up

• DOI: 10.1111/liv.13143
• Publication Date: 2016-04-10T18:24:30Z
• Authors: Aliya F. Gulamhusein; Brian D. Juran; Elizabeth J. Atki...; Bryan McCauley; Erik Schlicht; Konstantinos N. L...
• Keywords: First-degree relatives; Primary Biliary Cirrhosis; Proband

Abstract Background & Aims Primary biliary cirrhosis ( PBC ) is characterized by chronic cholestasis and disease‐specific antimitochondrial antibodies AMA ). A high prevalence of s in first‐degree relatives FDR s) probands has been reported, although the natural history such patients not described. We aimed to assess risk developing + with . Methods First‐degree recruited Mayo Clinic Genetic Epidemiology Registry Biorepository were followed for disease onset after recruitment. Development was ascertained via self‐report during a telephone interview and/or proband report on questionnaire. Chi‐squared test t ‐test used differences between categorical continuous variables respectively. mixed‐effects model change biochemical profiles over time. Results Forty 423 − subjects included median 8.9 8.4 years Overall, 3% n = 15) diagnosed , had higher than (24% vs. 0.7%, P < 0.01). However, among undiagnosed s, only 4% 1) 0.4% 0.17) follow‐up period. None normal alkaline phosphatase at baseline developed follow‐up. Conclusions Our results suggest low time particularly those without evidence baseline. These data are useful counselling reassuring their overall favourable prognosis.