A0485 - Prevalence of Diagnosed Primary Biliary Cholangitis and Associated Conditions in the United States: Results From a National Electronic Patient Database

Sunday, October 23, 2022

5:00 PM – 7:00 PM ET

Location: Crown Ballroom

Presenting Author(s)

Khaled Alsabbagh Alchirazi, MD

Cleveland Clinic
Cleveland, OH

Khaled Alsabbagh Alchirazi, MD¹, Abdul Mohammed, MD¹, Nour Azzouz, MD², Ashraf Almomani, MD³, Sobia Laique, MD¹, Szabo Gyongyi, MD, PhD⁴, Jaividhya Dasarathy, MD⁵, Amy Attaway, MD¹, Nicole Welch, MD¹, Srinivasan Dasarathy, MD¹
¹Cleveland Clinic, Cleveland, OH; ²University of Aleppo, Cleveland, OH; ³Cleveland Clinic Foundation, Cleveland, OH; ⁴Beth Israel Deaconess Medical Center, Boston, MA; ⁵MetroHealth, Cleveland, OH

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) (table1). PBC patients were at higher odds (Table 1) 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 (table 2).

Discussion: 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.

Figure: Table 2: 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.

	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

Table: Table1: Baseline Characteristic of Patients with Primary Biliary Cholangitis and Control Group. 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.

Disclosures:

Khaled Alsabbagh Alchirazi indicated no relevant financial relationships.

Abdul Mohammed indicated no relevant financial relationships.

Nour Azzouz indicated no relevant financial relationships.

Ashraf Almomani indicated no relevant financial relationships.

Sobia Laique indicated no relevant financial relationships.

Szabo Gyongyi: Generon – Consultant. Glympse – Advisory Committee/Board Member, Stock Options. Merck – Consultant. Novartiis – Advisor or Review Panel Member. Pfizer – Advisor or Review Panel Member. satellite bu=io – Consultant, Stock Options. Surrozen – Consultant. terraFirma – Advisor or Review Panel Member. Ventyx – Consultant, Stock Options.

Jaividhya Dasarathy indicated no relevant financial relationships.

Amy Attaway indicated no relevant financial relationships.

Nicole Welch indicated no relevant financial relationships.

Srinivasan Dasarathy indicated no relevant financial relationships.

Khaled Alsabbagh Alchirazi, MD¹, Abdul Mohammed, MD¹, Nour Azzouz, MD², Ashraf Almomani, MD³, Sobia Laique, MD¹, Szabo Gyongyi, MD, PhD⁴, Jaividhya Dasarathy, MD⁵, Amy Attaway, MD¹, Nicole Welch, MD¹, Srinivasan Dasarathy, MD¹. A0485 - Prevalence of Diagnosed Primary Biliary Cholangitis and Associated Conditions in the United States: Results From a National Electronic Patient Database, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.