S1252 Prevalence of Diagnosed Primary Biliary Cholangitis and Associated Conditions in the United States: Results From a National Electronic Patient Database

Abstract

Introduction: Primary biliary Cholangitis (PBC) is an autoimmune disease that is associated with a number of autoimmune disorders. PBC is associated with liver and non-liver organ co-morbidities that contribute to clinical outcomes. Several studies have suggested an increase in the incidence and prevalence of PBC, although this has not been assessed in a large cohort and has not been consistent across all epidemiologic studies. We determined the nationwide prevalence of PBC and its association with other autoimmune and non-liver related conditions in the United States (US). Methods: A database (Explorys Inc, Cleveland, OH) of electronic health record data from 26 major integrated US healthcare systems was used. Based on Systematized Nomenclature of Medicine – Clinical Terms (SNOMED-CT), patients (age >18 years) with a diagnosis of PBC between 1999 to present were identified. Data were analyzed in patients with/without a diagnosis of PBC. The prevalence of associated diseases was compared in in the 2 groups, and statistical univariate binary logistic model was performed. Results: Of the 70,383,890 individuals in the database, we identified 11,070 cases of PBC, with a prevalence of 15.7/100,000 persons (0.02%). Prevalence was higher in females than males [odds ratio (OR) 32.84; 95% Confidence Interval (CI) 30.50-35.37, p < 0.0001], adults (≥ 65 years) versus adult (18-65 years) (OR) 2.18; 95% CI 2.07-2.30, p < 0.0001) and whites versus non-whites (OR 14.54; 95% CI 13.63-15.51, p < 0.0001) (Table). PBC patients were at higher odds (Table) of co-morbidities including ischemic heart disease, cerebrovascular accident, congestive heart failure and sarcopenia when compared to non-PBC patients (p < 0.0001). PBC patients were more likely than non-PBC patients to have primary sclerosing cholangitis ( OR 1061.43) autoimmune hepatitis (OR 537.93), Type 1 diabetes mellitus (OR 240.58), ulcerative colitis (OR 15.25), Crohn’s disease (OR 7.84), celiac disease (OR 8.99), autoimmune thyroiditis (OR 7.69), systemic lupus erythematosus (OR 14.66) and other autoimmune disorders (Figure). Conclusion: Our large nationwide analysis study demonstrates higher prevalence of PBC in the US than previously reported, which is likely a result of diagnosis at an early stage and prolonged survival that may be related to improved management. The high association between PBC and other autoimmune conditions suggest shared common pathogenesis of immune-mediated destruction of end organs in genetically susceptible individuals. Table 1. - Baseline Characteristic of Patients with Primary Biliary Cholangitis and Control Group PBC n=11,070 (%) No PBC n=70,372,820 (%) OR (95%CI) p-value Demographics Age 18-65 4,490(41%) 47,940,720(68%) 0.32(0.31-0.33) <0.0001 Age > 65 6,620(60%) 21,251,790(30%) 3.44(3.31-3.57) <0.0001 Male 1,610(15%) 31,420,380(45%) 0.21(0.20-0.22) <0.0001 Female 9,390(85%) 38,453,360(55%) 4.64(4.41-4.89) <0.0001 Caucasian 8,770(79%) 37,743,080(54%) 3.30(3.15-3.45) <0.0001 Comorbidities T2DM 2,810(25%) 5,650,420(8%) 3.90(3.73-4.07) <0.0001 HTN 1,950(18%) 3,493,560(5%) 4.09(3.89-4.29) <0.0001 HLD 5,940(54%) 11,779,380(17%) 5.76(5.55-5.98) <0.0001 Tobacco use 2,220(20%) 6,486,950(9%) 2.47(2.36-2.59) <0.0001 Hypothyroidism 3,300(30%) 4,049,030(6%) 6.96(6.68-7.25) <0.0001 Ischemic Heart Disease 1,240(11%) 2,496,690(4%) 3.43(3.23-3.64) <0.0001 CHF 1,070(10%) 1,834,510(3%) 4.00(3.75-4.26) <0.0001 Sarcopenia 4,910(44%) 5,480,850(8%) 9.44(9.09-9.80) <0.0001 Alcohol abuse 320(3%) 1,090,250(2%) 1.89(1.69-2.11) <0.0001 Univariate analysis used to calculate OR, OR; odds ratio, CI; confidence interval, T2DM;Type 2 Diabetes Miletus, HTN; Hypertension, HLD; Hyperlipidemia, CHF; Congestive Heart Failure. PBC; Primary Biliary Cholangitis. Figure 1.: Primary Biliary Cholangitis Associated Autoimmune Disorders. Univariate analysis used to calculate OR, OR; odds ratio, CI; confidence interval, PSC; Primary Sclerosing Cholangitis, AIH; Autoimmune Hepatitis, T1DM; Type 1 Diabetes Miletus, AIHA; Autoimmune Hemolytic Anemia, UC; Ulcerative Colitis, CD; Crohn's Disease, SLE; Systemic Lupus Erythematosus, ITP; Idiopathic Thrombocytopenia Purpura, AIT; Autoimmune Thyroiditis, RA; Rheumatoid Arthritis, MG; Myasthenia Gravis.