Lu M, Li J, Haller IV, Romanelli RJ, VanWormer JJ, Rodriguez CV, Raebel MA, Boscarino JA, Schmidt MA, Daida YG, Sahota A, Vincent J, Bowlus CL, Lindor K, Rupp LB, Gordon SC; FOLD Investigators., Clin Gastroenterol Hepatol. pii: S1542-3565(17)31243-0. doi: 10.1016/j.cgh.2017.10.018. [Epub ahead of print], 2017 Oct 21
BACKGROUND & AIMS: Reported prevalence of primary biliary cholangitis (PBC) varies widely. Demographic features and treatment patterns are not well characterized in the United States (US). We analyzed data from the Fibrotic Liver Disease (FOLD) consortium, from 11 geographically diverse health systems, to investigate epidemiologic factors and treatment of PBC in the US.
METHODS: We developed a validated electronic health record-based classification model to identify patients with PBC in the FOLD consortium database, from 2003 through 2014. We used multivariable modeling to assess the effects of factors associated with PBC prevalence and treatment with ursodeoxycholic acid (UDCA).
RESULTS: We identified 4,241 PBC cases among over 14.5 million participants in the FOLD consortium health systems, followed for a median 5 years. The classification model identified patients with PBC with an area under the receiver operating characteristic curve value of 93%, with 94% sensitivity and 87% specificity. The average patient age at diagnosis was 60 years; 21% were Hispanic, 8% were African American, and 7% were Asian American/American Indian/Pacific Islander. Half of the cohort (49%) had increased levels of alkaline phosphatase, and overall, 70% were treated with UDCA. The estimated 12-year prevalence of PBC was 29.3 per 100,000 persons. Adjusted prevalence values were highest among women (42.8 per 100,000), White patients (29.6 per 100,000), and patients 60-70 yrs old (44.7 per 100,000). Prevalence was significantly lower among men and African Americans (10.7 and 19.7 per 100,000, respectively); men and African Americans were also less likely to receive UDCA treatment than women or Whites (odds ratios, 0.6 and 0.5, respectively; P<.05).
CONCLUSION: In an analysis of a large cohort of patients with PBC receiving routine clinical care, we observed significant differences in PBC prevalence and treatment by sex, race, and age.