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Autoimmunity Reviews 19 (2020) 102423

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Autoimmunity Reviews 19 (2020) 102423 Autoimmunity Reviews 19 (2020) 102423 Contents lists available at ScienceDirect Autoimmunity Reviews journal homepage: www.elsevier.com/locate/autrev Comparative United States autoimmune disease rates for 2010–2016 by sex, geographic region, and race T ⁎ Melissa H. Roberts , Esther Erdei University of New Mexico, College of Pharmacy, Albuquerque, NM, USA ARTICLE INFO ABSTRACT Keywords: Purpose: AIDs may disproportionately impact specific racial groups, but autoimmune (AID) prevalence in- Autoimmune disease formation by minority racial group is sparse for many AIDs. The objective of this analysis was to supplement Prevalence previously published AID prevalence rates by providing information on race rate ratios (minority race popula- North American tions compared to Caucasian populations) in the United States. Preliminary to estimating race rate ratios, Minority groups contemporary US-specific, health care utilization-based AID prevalence rates and female-to-male ratios were estimated and compared to previously published AID prevalence rates. Methods: We used a large national electronic medical record database of 52 million individuals to estimate age- adjusted direct standardized rates for 22 AIDs for 2010 through 2016 by gender, race, and US census division. These were compared to previously published estimates. Results: Female-to-male ratios were comparable with published studies. Almost all observed Multiracial AID rates were significantly higher than Caucasian rates, as well as 9 of 22 AID rates observed among Native Americans and 8 of 22 AID rates estimated among African-American patients. Regional variation was noted: highest African-American systemic lupus erythematosus rates were observed in the West North Central and South Atlantic divisions, highest African-American multiple sclerosis rates in the South Atlantic and Pacific divisions, and highest Native American rheumatoid arthritis rates in the West North Central, Mountain, and Pacific divisions. Conclusions: Substantial AID heterogeneity exists by race and by geographic area. An important research area is further exploring factors related to heterogeneity such as potential interactions between genetic susceptibility and environmental factors. 1. Introduction viruses, and toxic levels of drug and metal exposures [4]. Additionally, multifaceted interactions between environmentally induced cellular Autoimmune disease (AID) is a classification of pathological and and molecular processes and genetic susceptibility may play a role [5]. molecular features encompassing medical conditions that are complex, Overall, AID incidence worldwide is increasing, particularly in in- chronic, and occasionally life-threatening. Conditions having auto- dustrial countries like the United States (US) [6]. Collectively, AIDs are immune origins have increased over the years, and > 80 distinct AIDs estimated to impact from 5 to 8% of Americans or 15 to 20 million currently exist [1]. A heterogeneous group of diseases, AIDs affect a individuals, depending on the number of AIDs included in estimates range of organ systems, with some AIDs affecting a single organ and [2,4]. Because many AIDs are diagnosed in children and young adults, others affecting multiple systems. A majority of AIDs have a clear and are chronic lifetime conditions, AIDs pose a major economic and adaptive immune response to an autoantigen, but not all have a well- societal burden. Pharmaceutical therapy can be a substantive portion of established autoimmune etiology [2,3]. Factors associated with higher direct medical care cost, and the National Institutes of Health has es- risk of AIDs are genetic features, lifestyle characteristics (e.g., nutrition timated annual direct health care costs for AIDs to be in the range of and smoking) and environmental exposures that include bacteria, $100 billion [7]. Abbreviations: AID, autoimmune disease; CDC, Centers for Disease Control and Prevention; CI, confidence interval; CIPD, chronic inflammatory demyelinating polyneuropathy; DM1, diabetes mellitus type 1; EMR, electronic medical record; HF, Health Facts®; MS, multiple sclerosis; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; US, United States ⁎ Corresponding author at: University of New Mexico College of Pharmacy, MSC09 5360, 1 University of New Mexico, Albuquerque, NM 87131-0001, USA. E-mail addresses: [email protected] (M.H. Roberts), [email protected] (E. Erdei). https://doi.org/10.1016/j.autrev.2019.102423 Received 19 June 2019; Accepted 23 June 2019 Available online 14 November 2019 1568-9972/ © 2019 Elsevier B.V. All rights reserved. M.H. Roberts and E. Erdei Autoimmunity Reviews 19 (2020) 102423 AID burden can disproportionately fall to women as AIDs are more census), standardized age-adjusted rate ratios for females-to-males and prevalent among females [8,9]. AIDs may also disproportionately im- minority racial groups vs Caucasians, and 95% confidence intervals pact specific racial groups, but sparse information exists on minority (95% CI) were calculated using SAS procedure STDRATE [13]. group rates in the US. While the Centers for Disease Control and Pre- This study was considered exempt by the University's Institutional vention (CDC) has reported that for systemic lupus erythematosus Review Board. Data analyses were conducted using SAS (Cary, NC (SLE), “minority and ethnic groups, including blacks/African Amer- version 9.4) statistical software. icans, Hispanics/Latinos, Asians, and American Indians/Alaska Natives, are affected more than whites” [10], minimal epidemiological data has 3. Results been published concerning other complex autoimmune conditions. Obtaining reliable estimates of AID prevalence is complicated by not Over the 7-year period, among 52,246,831 unique individuals, only the difficulty in obtaining a definitive diagnosis for many AIDs, but 98.5% (n = 51,463,648) had age information, 93.0% (n = 48,609,646) also by the need for large study populations given the low prevalence of had sex and age information, and 75.6% (n = 39,516,604) had race and many AIDs. National surveys exist, for example those by the CDC [11], age information. Individuals identified as Asian or Pacific Islander were to collect information about more common AIDs like rheumatoid ar- combined into one category (Asian/Pacific Islander) due to small thritis (RA), but most rely on respondents to accurately self-report numbers for many census divisions. The overall race distribution of the morbidities. HF study population compared favorably with the US 2010 census In 2012 Hayter and Cook provided an updated assessment regarding population (Table 1). the prevalence, manifestations, and pathogenesis of 81 AIDS based on a Fig. 2 summarizes observed HF AID rates and comparison rates from review of US and worldwide clinical and epidemiological studies pub- Hayter and Cook [2]. Among observed rates, 14 of 22 were < 20 per lished 2010 and earlier [2]. Included were estimates for females as a 100,000: acquired hemophilia A, alopecia areata, autoimmune hemo- percentage of those with an AID. Heterogeneity in rates between racial lytic anemia, autoimmune hypoparathyroidism, autoimmune neu- groups was not discussed in Hayter and Cook's review [2], and in tropenia, chronic inflammatory demyelinating polyneuropathy (CIDP), general disparities for minority groups have been understudied in re- dermatitis herpetiformis, Guillain-Barre syndrome, immune thrombo- gard to AIDs. The objective of our analysis was to supplement the cytopenic purpura, myasthenia gravis, polymyositis/dermatomyositis, prevalence information provided by Hayter and Cook by providing primary biliary cirrhosis, scleroderma, and Sjögren's syndrome. For information on prevalence rates by race within the US. Using a large these 14 AIDs, 11 (78.6%) also had comparison rates < 20 per 100,000, electronic healthcare database of patients receiving care at facilities while the scleroderma comparison rate was slightly higher, and alo- across the US for the years 2010 to 2016, we estimated AID rate ratios pecia areata and immune thrombocytopenic purpura comparison rates by minority race group compared to Caucasian race within the US, both were substantially higher. The observed rate for Addison's disease (95% overall and by geographic area (census division). Preliminary to our CI: 21.3, 22.0) was close to the comparison rate range of 5 to 19. The main objective, we generated contemporary US-specific, health care celiac disease observed rate (95% CI: 20.3, 21.1) was substantially utilization-based prevalence rates for AIDs and female-to-male ratios lower than the comparison rate range of 600 to 800 per 100,000. and compared those rates and ratios to the Hayter and Cook estimates. Six AIDs had observed rates of ≥50 per 100,000 (grouped at the bottom of Fig. 2). Of these, two observed rates overlapped with com- 2. Methods parison rates: multiple sclerosis (MS) and ulcerative colitis. Two ob- served rates were higher than comparison rates: SLE and Crohn's dis- We utilized Cerner Health Facts® (HF) electronic medical record ease, while 2 observed rates were much lower: diabetes mellitus type 1 (EMR) data (Cerner HealthFacts®, Cerner Corp., Kansas City, MO), (DM1) and RA. which contains de-identified EMR data for approximately 16 million Fig. 3 summarizes observed and comparison female-to-male rate unique patients annually from over 600 hospitals and clinics in the US ratios. Observed and comparison ratios overlapped for 5 AIDs: auto- that use the Cerner medical record system and for which a data
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