Abstract
Objective
There are no estimates of the prevalence of giant cell arteritis (GCA) or polymyalgia rheumatica (PMR) in a US population in the current millennium. The purpose of this study was to estimate the 2015 prevalence of GCA and PMR in a US population-based setting.
Methods
Cohorts of incident GCA in 1950–2009 and incident PMR in 1970–2014 were previously identified among residents of Olmsted County, Minnesota. Prevalence estimates were obtained from the age-, sex- and calendar year-specific incidence rates and adjusted to the US white 2010 population aged ≥50 years. Confidence intervals [CI] were computed using bootstrap sampling.
Results
There were 248 incident cases of GCA in 1950–2009 and 790 cases of PMR in 1970–2014. The overall age- and sex-adjusted prevalence rate of GCA on January 1, 2015 was 204 (95% CI: 161–254) per 100,000 population aged ≥50 years. GCA prevalence rate in women was 304 (95%CI: 229–375) and in men was 91 (95%CI: 46–156) per 100,000 population. The overall age and sex adjusted prevalence rate of PMR on January 1, 2015 was 701 (95%CI: 651–750) per 100,000 population. PMR prevalence rate in women was 870 (95%CI: 787–957) and in men was 508 (95%CI: 426–589) per 100,000 population.
Conclusion
Prevalence rates of GCA and PMR in 2015 are similar to previously published prevalence estimates in the late 1990s. The health burden of these diseases among older persons continues to be substantial and reflects the need for continued efforts to better manage it.
Keywords: polymyalgia rheumatic, giant cell arteritis, epidemiology, prevalence
Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory disorders that occur almost exclusively in persons aged 50 years or older. Both diseases share many similarities, including increasing incidence with advancing age, female predominance, and similar geographical distribution.(1, 2) There is considerable overlap in the clinical features and epidemiology of PMR and GCA. Features of PMR occur in 40–60% of patients with GCA at diagnosis, and 16–21% of patients with PMR have GCA.(1)
PMR occurs at a frequency that is 3 to 10 times that of GCA.(3) Women have a higher lifetime risk for developing PMR (2.4%) than do men (1.7%).(3) The highest incidence rates are seen in northern latitudes, in Scandinavian countries and people of northern European descent.(1, 4) Similar to PMR, GCA is more common in these populations in the Northern hemisphere. The lifetime risk for GCA is about 1.0% for women and 0.5% for men.(3)
Although both diseases are associated with considerable disease and treatment related morbidities, most studies have reported either minimal or no increased risk for premature mortality from PMR and GCA.(5–7) Hence, prevalence rates and population health burdens are correspondingly high.
For PMR, prevalence estimates range from 20 per 100,000 in Turkey to 1530 per 100,000 in the UK.(8, 9) Using the Olmsted County age/sex prevalence rates and the corresponding 2005 estimates from the Census Bureau, Lawrence et al. calculated the prevalence of PMR in 2000 to be 739 per 100,000, and estimated that 711,000 Americans have PMR.(10) Available estimates of GCA prevalence range from 1.5/100,000 in Japan to 250/100,000 in the UK.(11, 12)
Again using the Olmsted County age/sex prevalence rates and the corresponding 2005 estimates from the Census Bureau, Lawrence et al. calculated the prevalence of GCA to be 204/100,000 and estimated that 228,000 Americans have GCA.(10)
This study was undertaken to develop a clearer contemporary picture of the frequency of these diseases in a susceptible population in the US.
Materials and Methods
Study population
The Olmsted County, Minnesota population is well-suited for epidemiologic investigation. The Rochester Epidemiology Project (REP) medical record-linkage system provides complete access to comprehensive medical records, both inpatient and outpatient, from all local healthcare providers, including the Mayo Clinic, the Olmsted Medical Center and their affiliated hospitals, local nursing homes and the few private practitioners, of all Olmsted County residents for over six decades.(13)
All Olmsted County residents diagnosed with incident GCA from January 1, 1950 to December 31, 2009 were previously identified. (6, 14) The diagnosis was based on 1990 American College of Rheumatology criteria for classification of GCA.(15) Patients aged ≥50 years with elevation of erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) and computed tomography/angiography, magnetic resonance arteriography or positron emission tomography evidence of large vessel vasculitis involving the ascending aorta and/or its branches were also included.
Similarly, all Olmsted County residents diagnosed with incidence PMR from January 1, 1970 to December 31, 2014 were previously identified.(7, 16) Patients were included if they fulfilled European League Against Rheumatism/American College of Rheumatology provisional classification criteria for PMR.(17)
Approval for this study was obtained from the institutional review boards of the Mayo Clinic and Olmsted Medical Center.
Statistical Methods
The prevalence of GCA and PMR on January 1, 2015 were estimated mathematically using the incident cohorts. For GCA, annual incidence rates between 2010 and 2014 were projected using the overall annual incidence rate for the 2009 time period. Prevalence can be easily estimated from incidence when three conditions are met: 1) the disease is not associated with any excess mortality; 2) there are no important calendar time trends; and 3) migration in or out of the census population is independent of disease status. In this case the first and third conditions were satisfied, but not the second condition, as there was a slight increase in the incidence rates of PMR throughout the 1970–2014 time period. In GCA, the incidence rates rose from 1950 to 1980 and were stable thereafter. Therefore, it was necessary to use a cohort method to estimate prevalence from incidence for both GCA and PMR.(18) The method involves applying age-, sex- and calendar year-specific incidence rates of disease and mortality rates from life tables to a hypothetical population to yield estimates of prevalence. Confidence intervals for the prevalence estimates were obtained using bootstrap methods. The estimated number of persons in the US with GCA and PMR on January 1, 2015 were estimated by applying the age- and sex- specific US population counts from the US Census Bureau to the estimated prevalence rates. Analyses were performed using SAS version 9.4 (SAS Institute, Cary, NC, USA) and R 3.1.1 (R Foundation for Statistical Computing, Vienna, Austria).
Results
A total of 248 incident cases of GCA were diagnosed in 1950–2009 and 760 cases of PMR were diagnosed in 1970–2014. The overall age- and sex-adjusted prevalence rate of GCA on January 1, 2015 was 204 (95% CI: 161–254) per 100,000 population aged ≥50 years (table 1). The prevalence rate of GCA among women was 304 (95%CI: 229–375) and among men was 91 (95%CI: 46–156) per 100,000 population aged ≥50 years. Prevalence rates of GCA rose precipitously from age 50–54 year to age 90+ years in both sexes (figure 1).
Table 1.
Prevalence rates for giant cell arteritis
| Author | Country | Year | Age, years | GCA criteria | GCA prevalence rate per 100,000 (95% confidence interval) | ||
|---|---|---|---|---|---|---|---|
| Overall | Female | Male | |||||
| Current Study | USA | 2015 | ≥ 50 | ACR 1990 | 204 (161, 254) | 304 (229, 375) | 91 (46, 156) |
| Catanoso(19) | Italy | 2012 | ≥ 50 | Positive TAB; ACR 1990 | 87.9 (75.8, 101.4) | ||
| Lawrence(10) | USA | 2000 | ≥ 50 | ACR 1990 | 228 (192, 268) | 344 | 200 |
| Yates(12) | UK | 2013 | ≥ 55 | Jones/Hazleman | 250 (110, 390) | 330 (120, 550) | 160 (0, 320) |
| Kobayashi(11) | Japan | 1997 | ≥ 50 | ACR 1990 | 1.5 | ||
| Gonzalez-Gay(20) | Spain | 1991 | ≥ 50 | Hunder | 60 | ||
| Herlyn(21) | Germany | 2006 1994 | ≥ 50 | ACR 1990 | 44.0 (39.9, 48.1) 24.0 (1.64, 31.5) |
61.2 (56.4, 66.0) | 21.9 (19.0, 24.8) |
| Pamuk(8) | Turkey | ≥ 50 | ACR 1990 | 20 (16, 24) | |||
| Andrianakos(22) | Greece | 1996 | ≥ 50 | ACR 1990 | 80 (10, 150) | ||
Abbreviations: ACR, American College of Rheumatology; GCA, giant cell arteritis; TAB, temporal artery biopsy
Figure 1.
Prevalence of giant cell arteritis (black lines) and polymyalgia rheumatica (grey lines) in Olmsted County, Minnesota on January 1, 2015 overall (solid lines) and by sex (dashed lines depict rates for females and dotted lines depict rates for males)
The overall age and sex adjusted prevalence rate of PMR on January 1, 2015 was 701 (95%CI: 651–750) per 100,000 population aged ≥50 years (table 1). The prevalence rate of PMR among women was 870 (95%CI: 787–957) and among men was 508 (95%CI: 426–589) per 100,000 population aged ≥50 years. Prevalence rates of PMR also rose precipitously from age 50–54 year to age 90+ years in both sexes (figure 1).
Based on 2015 US population estimates, an estimated 214,000 persons aged ≥50 years in the US have GCA (168,000 women and 46,000 men) and 745,000 persons have PMR (486,000 women and 259,000 men aged ≥50 years).
Discussion
Both PMR and GCA are relatively common diseases of older persons. The actual population burden varies according to population and geography, but both occur most frequently in populations of northern European ancestry. In this population-based study with records dating from 1950 to 2009 for GCA, and 1970 to 2014 for PMR, the overall age- and sex-adjusted prevalence rate of GCA in persons over the age of 50 years on January 1, 2015 was 204 per 100,000, and for PMR, 701 per 100,000 population. Prevalence was about three times higher for PMR, and both diseases were two to three times more common in women, and both diseases are markedly more prevalent in persons of advanced age.
Because patients with PMR and GCA have normal or near-normal life expectancy compared to the general population, consistent with studies of incidence, the prevalence of PMR and GCA is higher in northern European populations. Annual incidence rates for PMR in persons aged 50 years and older range from 113 per 100.000 in Norway to 41–84 cases per 100,000 population in the United Kingdom (UK), Denmark and Sweden and 59 cases per 100,000 in Olmsted County, Minnesota, USA.(4) The annual incidence of GCA is also higher in northern European countries in persons over the age of 50 (about 20 per 100,000 population) than in southern European countries (less than 12 cases per 100,000) or in Hispanic and Asian populations.(4, 19) In the US population, GCA is the most frequent primary vasculitis with an incidence of 18 cases per 100,000 Olmsted County residents.(10)
The prevalence of PMR and GCA in this population is very similar to that reported in the UK, and considerably higher than that reported from southern Europe and non-European populations (tables 1 and 2).(8, 10–12, 19–24) Prevalence estimates in the current study have remained stable over the long period of population observation, although a recent slight increase in PMR incidence may increase future prevalence estimates.(7) Differences in prevalence estimates in these various cohorts are most likely due to differences in disease classification and diagnosis criteria used, as well as genetic and geographic factors.
Table 2.
Prevalence rates for polymyalgia rheumatica
| Author | Country | Year | Age, years | PMR Criteria | PMR prevalence rate per 100,000 (95% confidence interval) | ||
|---|---|---|---|---|---|---|---|
| Overall | Female | Male | |||||
| Current study | USA | 2015 | ≥ 50 | ACR/EULAR 2012 | 701 (651, 750) | 870 (787, 957) | 508 (426, 589) |
| Lawrence(10) | USA | 2000 | ≥ 50 | Doran | 739 (674, 808) | 925 | 532 |
| Yates(12) | UK | 2013 | ≥ 55 | Bird(25) Doran Jones/Hazleman | 910 (650, 1170) 1200 (900, 1500) 1530 (1200, 1870) |
1040 (650, 1420) 1440 (990, 1890) 1810 (1310, 2310) |
780 (430, 1120) 940 (560, 1320) 1220 (790, 1666) |
| Salaffi(23) | Italy | 2004 | ≥ 18 | Bird | 370 (290, 440) | ||
| Bernatsky(24) | Canada | 2006 | ≥ 45 | Claims data - urban rural |
755 (674, 850) 1004 (886, 1143) |
274 (220, 348) 381 (311, 468) |
|
| Pamuk(8) | Turkey | ≥ 50 | Chuang(26) | 20 (16, 24) | |||
| Andrianakos(22) | Greece | 1996 | ≥ 50 | Bird | 370 (220, 520) | ||
Abbreviations: ACR, American College of Rheumatology; EULAR, European League Against Rheumatism; PMR, polymyalgia rheumatica
This study is the first to report the prevalence PMR and GCA over a long period of time, using population data for up to 65 years for GCA and 45 years for PMR. A particular strength of this study is use of the Rochester Epidemiology Project record linkage system, through which all cases of PMR and GCA in Olmsted County, Minnesota were identified and verified by individual medical record review, which avoids referral bias within the study cohort. The ability to review the entire medical record of each study subject to collect information pertinent to the study avoids potential recall biases that might arise if data were collected in other ways, such as interviews or questionnaires.
A potential limitation of the retrospective study design may be incomplete documentation in the medical record regarding symptoms suggestive of PMR and GCA. Because the population of Olmsted County is predominantly Caucasian, results may not be generalizable to non-Caucasian individuals, however this population represents the cohort at highest risk of developing these diseases.
In conclusion, this first study of GCA and PMR prevalence in a US population in the current millennium revealed prevalence in 2015 to be comparable to the late 1990s. The population health burden of these diseases among older persons continues to be substantial and reflects the need for continued efforts to better manage it.
Acknowledgments
Funding: This study was made possible using the resources of the Rochester Epidemiology Project, which is supported by the National Institute on Aging of the National Institutes of Health under Award Number R01AG034676, and CTSA Grant Number UL1 TR000135 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
Footnotes
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Conflict of interest statement for all authors: The authors have no financial or non-financial potential conflicts of interest to declare.
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