Kex 260

Rheumatology 2017;56:19451961
RHEUMATOLOGY doi:10.1093/rheumatology/kex260
Advance Access publication 9 August 2017
Original article
The worldwide incidence and prevalence of systemic
lupus erythematosus: a systematic review of
epidemiological studies
Frances Rees1,2, Michael Doherty1, Matthew J. Grainge3, Peter Lanyon1,2 and
Downloaded from https://academic.oup.com/rheumatology/article-abstract/56/11/1945/4079913 by guest on 27 May 2019

Weiya Zhang1
Abstract
Objectives. The aim was to review the worldwide incidence and prevalence of SLE and variation with age,
sex, ethnicity and time.
Methods. A systematic search of MEDLINE and EMBASE search engines was carried out using Medical
Subject Headings and keyword search terms for Systemic Lupus Erythematosus combined with inci-
dence, prevalence and epidemiology in August 2013 and updated in September 2016. Author, journal,
year of publication, country, region, case-finding method, study period, number of incident or prevalent
cases, incidence (per 100 000 person-years) or prevalence (per 100 000 persons) and age, sex or ethnic
group-specific incidence or prevalence were collected.
Results. The highest estimates of incidence and prevalence of SLE were in North America [23.2/100 000
person-years (95% CI: 23.4, 24.0) and 241/100 000 people (95% CI: 130, 352), respectively]. The lowest
incidences of SLE were reported in Africa and Ukraine (0.3/100 000 person-years), and the lowest preva-
lence was in Northern Australia (0 cases in a sample of 847 people). Women were more frequently
affected than men for every age and ethnic group. Incidence peaked in middle adulthood and occurred
later for men. People of Black ethnicity had the highest incidence and prevalence of SLE, whereas those
with White ethnicity had the lowest incidence and prevalence. There appeared to be an increasing trend of
SLE prevalence with time.
CLINICAL
SCIENCE
Conclusion. There are worldwide differences in the incidence and prevalence of SLE that vary with sex,
age, ethnicity and time. Further study of genetic and environmental risk factors may explain the reasons
for these differences. More epidemiological studies in Africa are warranted.
Key words: incidence, prevalence, epidemiology, systemic lupus erythematosus, systematic review
Rheumatology key messages

. There is wide geographical variation in the reported incidence and prevalence of SLE.
. Males with SLE have an older peak age of incidence and prevalence compared with females.
. There appears to be a trend of increasing prevalence of SLE with time.
Introduction
Systemic Lupus Erythematosus (SLE) is a chronic autoim-
1
Division of Rheumatology, Orthopaedics and Dermatology, University mune disease with a varying clinical phenotype. It is
of Nottingham, 2Rheumatology Department, Nottingham University known to affect women more frequently than men, with
Hospitals NHS Trust and 3Division of Epidemiology and Public Health,
University of Nottingham, Nottingham, UK a ratio of approximately six women to every one man [1].
Submitted 5 January 2017; revised version accepted 6 June 2017 The aetiology of SLE is not fully understood, but both
Correspondence to: Frances Rees, Academic Rheumatology, The genetic predisposition and environmental triggers are
University of Nottingham, Room A27, Clinical Sciences Building, City believed to be involved [2]. Studying the epidemiology of
Hospital, Nottingham NG5 1PB, UK.
E-mail: [email protected] SLE allows us to identify and explore changes in potential
! The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: [email protected]
Frances Rees et al.
risk factors for the disease and allows planning of health incidence or prevalence rates reported were collected.
services in response to overall disease burden [3]. A Age-adjusted or standardized results were presented
review of the incidence and prevalence of SLE was last whenever available. PRISMA guidelines were used.
published in 2006 by Danchenko et al. [4] and found
marked disparities in incidence and prevalence world- Results
wide. This was attributed to both true geographical vari-
ation and variation in study design. It could be a result of Incidence
differences in the age and ethnic mix between popula-
Geography
tions, the definition of SLE used or, as found in some
studies in the same population, a change in the incidence Table 2 and Fig. 1A summarize the reported worldwide
and prevalence of SLE with time [1, 57]. The aim of this incidence estimates of SLE. Figure 1A uses the most

study was to review the current literature published on the recent estimates from Table 2. There was worldwide vari-
incidence and prevalence of SLE throughout the world. ation, with the highest incidence reported in North America
(23.2/100 000 person-years, 95% CI: 22.4, 24.0) [8] and the
lowest incidences reported in Africa (0.3/100 000 person-
Methods years) [9] and Ukraine (0.3/100 000 person-years, 95% CI:
0.0, 1.5) [10]. In general, European countries had a lower
A systematic literature review was undertaken. The search
incidence of SLE, whereas Asia, Australasia and the
strategy used both Medical Subject Headings (MeSH) and
Americas had a higher incidence. The most frequent meth-
keyword search terms for Systemic Lupus Erythematosus
ods for case-finding were local secondary care hospital-
combined with MeSH and keyword terms for incidence and
based outpatient lists or discharge registries, or National
epidemiology, followed by prevalence and epidemiology
Health Insurance databases.
(see supplementary Table S1, available at Rheumatology
Online, for search strategy). The databases searched were Age and sex
Ovid MEDLINE from 1946 to August 2013 and EMBASE In all studies reviewed, females had a higher incidence of
from 1974 to August 2013. All articles were downloaded SLE compared with males. The sex ratio ranged from 2:1
into Endnote software and were selected on the basis of [36] to 15:1 [46]. As an example, Somers et al. [31] esti-
title and then abstract for full review. Hand-searching of cit- mated the UK incidence to be 7.89/100 000 person-years
ations also occurred. Articles were included if they were (95% CI: 7.46, 8.31) for females compared with 1.53/
written in English or French language and were regarding 100 000 person-years (95% CI: 1.34, 1.71) for males.
humans. Exclusion criteria were review articles, conference This higher incidence in females remained true for every
proceedings, abstracts or editorials, articles in press, art- age group, although the ratios were smaller at both ex-
icles involving drug-induced lupus or neonatal lupus, and tremes of age.
those solely regarding paediatric patients or a subtype of In the majority of studies, there was a peak age of inci-
SLE, such as LN or discoid lupus. Searches were updated dence before declining. In females, the peak age ranged
in September 2016. Table 1 shows the number of articles from the third to seventh decades of life. For males, the
retrieved from each database in August 2013 and the add- peak incidence was usually later, in the fifth to seventh
itional articles added in September 2016. decades. Three selected studies taken from three differ-
Information on author, journal, year of publication, coun- ent geographical regions demonstrate this in Fig. 2A.
try, region, case-finding method, study period, number of
incident or prevalent cases, incidence (per 100 000 person- Ethnicity
years) or prevalence (per 100 000 persons) was collected In studies that reported differences between ethnic groups
by F.R. In addition, any age, sex or ethnic group-specific [1, 8, 21, 29, 33, 35, 37, 41, 42, 58, 59], incidence rates
TABLE 1 Summary of literature search
Number of art-
icles selected
for inclusion
Number of art- after reading Number of add-
icles selected the full text art- itional articles
Number of for review on icle, including selected on
articles after the basis of additional art- updated search
Number of art- removing title and icles found by in September
Search term Database icles retrieved duplicates abstract hand searching 2016
Incidence Medline 542 1617 76 46 11

Embase 1175
Prevalence Medline 929 2744 92 76 14
Embase 2290
1946 www.rheumatology.oxfordjournals.org
TABLE 2 Worldwide incidence of SLE
Incidence per 100 000

Number of person-years (95% CI)
Continent Country References Region Case-finding method incident cases [study year]
Europe Denmark Voss et al. [5] Funen Hospital and community records 127 1.0 (0.3, 2.9)a [1980]
3.6 (2.0, 6.1)a [1994]
Laustrup et al. [11] Funen Hospital and community records 35 1.0 (0.3, 2.7)
www.rheumatology.oxfordjournals.org
Hermansen et al. [12] National National patient registry 1644 2.35 (2.24, 2.49)
France Arnaud et al. [13] National National health insurance database 1931 3.32
Finland Elfving et al. [14] Northern Savo Hospital and community records 7 3.6 (3.0, 4.2)a
Greece Alamanos et al. [15] North-west Hospital records 178 1.9 (1.5, 2.3)a
Iceland Gudmundsson et al. [16] National Hospital registers 76 3.3
Italy Govoni et al. [17] Ferrara Hospital records 2000: 7 2.0
2001: 4 1.2
2002: 9 2.6
Tsioni et al. [18] Valtrompia Hospital and community records 9 2.0 (0.9, 3.8)
Norway Nossent [19] North Hospital records 83 2.9 (2.4, 3.3)a
Eilertsen et al. [20] North Hospital records 58 3.0 (2.0, 4.0)
Lerang et al. [21] Oslo Hospital records 116 3.0 (2.4, 3.5)
Spain López et al. [22] Asturias Hospital records 116 2.2 (1.8, 2.5)
Gómez et al. [23] Asturias Hospital records 1.9 (1.1, 2.7)
Alonso et al. [24] Lugo Hospital records 150 3.6 (3.0, 4.2)a
Sweden Leonhardt [7] Malmö Hospital records 16 1.0a
Eyrich et al. [25] Halmstad Hospital records 41 1.8 [1957, 1964]
3.0 [1964, 1971]
Jonsson et al. [26] Lund and Orup Hospital and community records 39 4.0 (1.6, 6.4)a
Ståhl-Hallengren et al. [6] Lund and Orup Hospital and community records 41 4.8
Ingvarsson et al. [27] Lund and Orup Hospital and community records 55 2.8 (1.4, 4.2)
UK Hopkinson et al. [28] Nottingham Hospital records 23 4.0 (2.3, 5.6)a
Johnson et al. [29] Birmingham Hospital records 33 3.8 (2.5, 5.1)
Nightingale et al. [30] Whole UK CPRD 390 3.0 (2.7, 3.3)
Somers et al. [31] Whole UK CPRD 1638 4.7 (4.5, 4.9)a
Rees et al. [1] Whole UK CPRD 2740 4.9 (4.7, 5.1)
North America Canada Bernatsky et al. [32] Quebec Physician billing database 219 3.0 (2.6, 3.4)
Hospitalization database 203 2.8 (2.6, 3.0)
USA Siegel et al. [33] New York and Alabama Hospital records New York: 98 1.9
Alabama: 63 1.0
Fessel [34] San Francisco Hospital records 74 7.6
Hochberg [35] Baltimore Hospital records 302 4.6a
Michet et al. [36] Minnesota Hospital records and death certificates 25 1.8 (1.1, 2.5)a
McCarty et al. [37] Pennsylvania Community and hospital records 191 2.4 (2.1, 2.8)a
(continued)
1947
Worldwide incidence and prevalence of SLE

1948
TABLE 2 Continued
Incidence per 100 000

Frances Rees et al.
Number of person-years (95% CI)

Continent Country References Region Case-finding method incident cases [study year]
Uramoto et al. [38] Minnesota Hospital records 48 5.6 (3.9, 7.2)a

Naleway et al. [39] Wisconsin Medical records 44 5.1 (3.6, 6.6)a
Feldman et al. [8] Whole US Medicaid database 3490 23.2 (22.4, 24.0)
Furst et al. [40] Whole US Medical claims database 1557 7.2 (6.8, 7.7)a
Lim et al. [41] Georgia Georgia Lupus registry 267 5.6 (5.0, 6.3)a
Somers et al. [42] Michigan Medical records 399 5.5 (5.0, 6.1)a
Jarukitsopa et al. [43] Minnesota Rochester epidemiology project database 45 2.9 (2.0, 3.7)
Central America Caribbean Nossent [44] Curaçao Medical records 68 4.6 (0.4, 8.8)
Deligny et al. [45] Martinique Medical records 180 4.7 (2.5, 6.9)
Flower et al. [46] Barbados National hospital-based SLE registry 183 6.3 (5.4, 7.3)a
South America Argentina Scolnik [47] Buenos Aires Private medical care database 68 6.3 (4.9, 7.7)
Brazil Pereira Vilar et al. [48] Natal city Hospital records 43 8.7 (6.3, 11.7)
Nakashima et al. [49] Cascavel Medical records 14 4.8
Africa Zimbabwe Taylor et al. [9] Bulawayo and Harare Hospital records 22 0.3
Asia China Mok et al. [50] Hong Kong University hospital database 3.1
Kazakhstan Nasonov et al. [10] Semey Hospital records 4 1.3 (0.4, 3.4)a
Russia Nasonov et al. [10] Kursk and Yaroslavl Hospital records 12 1.2 (0.6, 2.1)a
Ukraine Nasonov et al. [10] Vinnitsa Hospital records 1 0.3 (0.0, 1.5)a
South Korea Shim et al. [51] National National Health Insurance database 1398 2.8 (2.72.9)a
Taiwan Chiu et al. [52] National National Health Insurance database 12 789 8.1
Kang et al. [53] National National Health Insurance database 758 3.3
Yu et al. [54] National National Health Insurance database 671 8.4 (7.7, 9.0)
Yeh et al. [55] National Catastrophic illness database 6675 4.9
See et al. [56] National National Health Insurance database 358 7.2 (6.5, 8.0)
Australasia Australia Anstey et al. [57] Northern Territory Hospital records 13 11
a
Age standardized. CPRD: UK Clinical Practice Research Datalink.
FIG. 1 The global incidence (A) and prevalence (B) of SLE (most recent estimates used)

were highest in Black populations and lowest in 29.6, 32.9)] and significantly higher than for Whites [18.0/
Caucasians. Asian and Hispanic ethnic groups were inter- 100 000 person-years (95% CI: 17.0, 19.0)] and Asians
mediate. For example, in the UK, Hopkinson et al. [59] [16.7/100 000 person-years (95% CI: 13.9, 20.0)]. In the
published race-specific incidence figures for Nottingham, same study, the incidence in Hispanics was 22.2/100 000
with Afro-Caribbeans highest at 31.9/100 000 person- person-years (95% CI: 20.4, 24.2). A study specifically
years, Asians 4.1/100 000 person-years and Whites 3.4/ focusing on native American Indians found that three
100 000 person-years. In North America, Native American tribes had a particularly high incidence of SLE, specifically
Indians also had higher incidence rates than the White the Crow, Arapahoe and Sioux tribes [60].
population. This was demonstrated in the study by
Feldman et al. [8], where the incidence in native Temporal trend
American Indians was 30.0/100 000 person-years (95% There were a number of studies that examined the same
CI: 22.5, 39.9), which was similar to that of Black or population at risk over time, allowing us to examine the
African Americans [31.2/100 000 person-years (95% CI: temporal trend (Fig. 3A). In the UK, Somers et al. [31]
www.rheumatology.oxfordjournals.org 1949
Frances Rees et al.
FIG. 2 The incidence (A) and prevalence (B) of SLE stratified by age and sex in the UK, USA and Taiwan

showed a small but non-significant increase in the inci- Michet et al. [36] for the period 195079, when the inci-
dence in females over the 10-year period 199099, but dence was 1.8/100 000 person-years (95% CI: 1.1, 2.5),
not with males. However, Rees et al. [1] found a statistic- followed by Uramoto et al. [38], who published data for
ally significant decline in incidence from 1999 to 2012 of 198092, when the incidence rate was 5.6/100 000
1.8% per year. In the County of Funen in Denmark, Voss person-years (95% CI: 3.9, 7.2), and finally, Jarukitsopa
et al. [5] looked at the time periods 198084, 198589 and et al. [43], who examined 19932005 and found the inci-
199094. The respective incidence rates were 1.0 (95% dence rate had declined to 2.9/100 000 person-years
CI: 0.6, 1.6), 1.1 (95% CI: 0.7, 1.7) and 2.5 (95% CI: 1.8, (95% CI: 2.0, 3.7).
3.3) per 100 000 person-years. Although not linear, there
was a significant increase from the first to the last 5-year Prevalence
period. Although this could be a true increase, from 1
January 1993 an additional data source was available, Geography
thus increasing the number of cases identified. The prevalence of SLE by country is summarized in Table
Alamanos et al. [15], in North-West Greece, showed an 3 and Fig. 1B. Figure 1B uses the most recent estimates
increasing trend from 1.41/100 000 person-years (95% from Table 3. The lowest prevalence was reported in a
CI: 0.99, 1.83) in 198286 to 2.19/100 000 person-years community study of 847 people in Yarrabah, North
(95% CI: 1.78, 2.60) in 19972001, but this was not stat- Queensland, Australia [61], where no cases were found.
istically significant. Finally, results from the Rochester The highest prevalence was in a national survey in the
Epidemiology project in Minnesota were published by USA [62], which reported a prevalence of 241/100 000
TABLE 3 Worldwide prevalence of SLE
Prevalence, per
Prevalent 100 000 (95% CI)
Continent Country References Study period Region Case-finding method cases [year of study]
Europe Denmark Voss et al. [5] 1 January 1995 Funen Hospital and community 84 22.2a
records
Laustrup et al. [11] 1 January 2003 Funen Hospital and community 109 28.3 (23.3,
records 34.2)
Eaton et al. [63] 31 October 2006 National National hospital patient 48
registry
Hermansen et al. [12] 31 Decmeber 2011 National National hospital patient 1887 45.2 (43.3,
registry 47.4)
Finland Helve [64] December 1978 National National hospital dis- 1427 28
charge database
France Arnaud et al. [13] 2010 National National Health Insurance 27 369 40.8a
database
Germany Brinks et al. [65] 2002 National National Health Insurance 845 36.7 (34.3,
database 39.3)
Greece Alamanos et al. [15] 31 December 2001 North-West Hospital records 193 38.1 (36.3,
39.9)a
Anagnostopoulos et al. [66] 2008 Central Postal survey 2 110 (110, 370)
Iceland Gudmundsson et al. [16] 197584 National Hospital registers 86 35.9a
Italy Benucci et al. [67] June 2002 Florence Community survey 23 71 (49, 92)a
Govoni et al. [17] 2002 Ferrara Hospital records 201 57.9
Sardu et al. [68] July 2009 Southern Sardinia Community records 81 (50, 124)
Tsioni et al. [18] 31 December 2012 Valtrompia Hospital and community 44 39.2 (28.5,
records 52.6)
Lithuania Dadoniene et al. [69] 2004 Vilnius Hospital records and 76 16.2 (12.7,
community survey 20.3)
Norway Nossent [19] 1996 North Hospital records 89 49.7 (44.3, 55)a
Eilertsen et al. [20] 2007 North Hospital records 114 64.1
Lerang et al. [21] 1 January 2008 Oslo Hospital records 238 52.8 (45.2,
58.4)
Spain López et al. [22] 31 December 2002 Asturias Hospital records 367 34.1 (30.6,
37.6)
Gómez et al. [23] December 2003 Asturias Hospital records 31.7 (28.3,
35.0)
Alonso et al. [24] 31 December 2006 Lugo Hospital records 150 17.5 (12.6,
24.1)a
Sweden Leonhardt [7] 1955 Malmö Hospital records 2.9
1958 4.5
1961 6.0
Nived et al. [70] 31 December 1982 Lund and Orup Hospital and community 61 39 (30, 48)
records
1951
(continued)

TABLE 3 Continued
1952
Prevalence, per
Ståhl-Hallengren et al. [6] 31 December 1986 Lund and Orup Hospital and community 121 42
31 December 1991 records 162 68
Frances Rees et al.
Simard et al. [71] 1 January 2010 National National patient register 7929 (46, 85)
Ingvarsson et al. [27] 31 December 2006 Lund and Orup Hospital and community 174 65
records
Turkey Çakır et al. [72] Havsa Community survey 10 57 (46, 70)a
UK Hochberg [73] 198182 Whole UK Community medical 20 6.5
record survey
Samanta et al. [74] 198689 Leicester Hospital records 50 26.1
Hopkinson et al. [28] 30 April 1990 Nottingham Hospital records 147 24.6 (20.6,
28.7)a
Johnson et al. [29] 1992 Birmingham Hospital records 242 27.7 (24.2,
31.2)
Gourley et al. [75] 1 August 1993 Northern Ireland Hospital records 408 25.4 (22.1,
28.7)a
Nightingale et al. [76] 199298 Whole UK CPRD 1538 25.0 (23.4,
26.7) [1992]
40.7 (37.6,
43.8) [1998]
Rees et al. [1] 19992012 Whole UK CPRD 1875 65.0 (62.1,
67.9) [1999]a
4413 97.0 (94.2,
99.9) [2012]a
North America Canada Peschken et al. [77] 1996 Manitoba Medical records 257 22.1 (13.2,
32.4)
Bernatsky et al. [32] 2003 Quebec Physician billing and hos- 3825 44.7 (37.4,
pitalization databases 54.7)a
USA Siegel et al. [58] 1959 New York Hospital records 5
Fessel [34] 1973 San Francisco Hospital records 64 50.8
Serdula et al. [78] 1975 Oahu, Hawaii Hospital records 81 15.3a
Michet et al. [36] 1 January 1980 Minnesota Hospital records 20 40.0 (23.5,
57.5)
Uramoto et al. [38] 1 January 1993 Minnesota Hospital records 122 (97, 217)a
Maskarinec et al. [79] 1989 Hawaii 454 41.8
Post et al. [80] 1996 California Postal survey 20 68.2
Balluz et al. [81] 1997 Arizona Hospital and community 20 103 (56, 149)
records
Ward [62] 198894 National US National health survey 40 241 (130, 352)
Naleway et al. [39] 2001 Wisconsin Medical records 64 78.5 (59.0,
98.0)a
Chakravarty et al. [82] 2000 California and Hospitalization databases California:
Pennsylvania 107.6 (106.1,
109.2)a
(continued)

TABLE 3 Continued
Prevalence, per
Pennsylvania:
149.5 (146.9,
152.2)a
Feldman et al. [8] 200004 National Medicaid database 34339 143.7 (142.2,
145.3)
Furst et al. [40] 200308 National Medical claims database 15396 81.1 (78.5,
83.6) [2003]
102.9 (100.4,
105.5) [2008]
Lim et al. [41] 2002 Georgia Georgia Lupus registry 1156 73.0 (68.9,
77.4)a
Somers et al. [42] 200204 Michigan Medical records 2139 72.8 (70.8,
74.8)a
Jarukitsopa et al. [43] 1 January 2006 Rochester, MN Rochester epidemiology 72 53.5 (41.1,
project database 65.9)
Central America Caribbean Nossent [44] 1 January 1990 Curaçao Medical records 69 47.6 (34.1,
51.1)
Deligny et al. [45] 1999 Martinique Medical records 245 64.2 (56.2,
72.2)
Molina et al. [83] 2003 Puerto Rico Private health insurance 877 159
database
Reyes-Llerena et al. [84] Havana, Cuba WHO-ILAR COPCORD 2 60 (10, 200)
study
Flower et al. [46] 31 October 2009 Barbados National hospital-based 226 84.1 (73.5,
SLE registry 95.8)
Mexico Peláez-Ballestas et al. [85] Five regions in WHO-ILAR COPCORD 60 (30, 100)a
Mexico study
South America Argentina Scolnik et al. [47] 1 January 2009 Buenos Aires Private medical care 75 58.6 (46.1,
database 73.5)
Brazil Rodrigues Senna et al. [86] Montes Claros City WHO-ILAR COPCORD 3 98 (20, 280)
study
Venezuela Granados et al. [87] 2011 Monagos WHO-ILAR COPCORD 3 70 (10, 200)
study
Asia China Wigley et al. [88] North (near Beijing) WHO-ILAR COPCORD North: 3 10
South (near study South: 1 20
Shantou)
Li et al. [89] Beijing Community survey 3 30 (0, 60)
India Malaviya et al. [90] Delhi Community survey 3 3.2 (0, 6.86)
Iran Davatchi et al. [91] September 2005 Tehran city WHO-ILAR COPCORD 3 40
study
Davatchi et al. [92] September 2006 Five villages WHO-ILAR COPCORD 1 60 (6, 670)
in NW Iran study
(continued)
1953

TABLE 3 Continued
1954
Prevalence, per
Kazakhstan Nasonov et al. [10] 31 December 2010 Semey Hospital records 52 17.3 (12.9,
22.6)a
Frances Rees et al.
Malaysia Wang et al. [93] 197490 Kuala Lumpur Hospital records 539 43
Pakistan Farooqi et al. [94] North WHO-ILAR COPCORD 1 50
study
Russia Nasonov et al. [10] 31 December 2010 Kursk and Yaroslavl Hospital records 79 7.7 (6.1, 9.7)a
South Korea Ju et al. [95] 200406 National National Health Insurance 900011000 18.8, 21.7
database
Shim et al. [51] 200610 National National Health Insurance 10080 20.6 (20.2,
database 21.0) [2006]
13316 26.5 (26.0,
27.0) [2010]
Taiwan Chou et al. [96] Cu-Tien Community survey 1 33
Chiu et al. [52] 200007 National National Health Insurance 15463 42.2 [2000]
database 67.4 [2007]
Kang et al. [53] 31 December 2005 National National Health Insurance 15753 69.3
database
Yu et al. [54] 2000 National National Health Insurance 356 37.0 (10.0,
database 41.0)
Yeh et al. [55] 2003 National Catastrophic illness 133488 97.5
2008 database
See et al. [56] 2005 National National Health Insurance 435 43.5 (39.4,
database 47.6)
Ukraine Nasonov et al. [10] 31 December 2010 Vinnitsa Hospital records 45 12.2 (8.9,
16.4)a
Australasia Australia Anstey et al. [57] 1January 1991 Northern Territory. Hospital records 22 52
Grennan et al. [97] 1993 Queensland Sydney Hospital records Queensland: 20 89
Sydney: 3 13
Bossingham [98] 1 August 1996 to Far North Hospital records 108 45.3
31 August 1998 Queensland
Minaur et al. [61] January 2002 Yarrabah, North WHO-ILAR COPCORD 0 0
Queensland study
New Zealand Meddings et al. [99] Dunedin Hospital records 16 14.7
Hart et al. [100] 1980 Auckland Hospital records 136 17.6a
a
Age standardized. CPRD: clinical practice research datalink; WHO-ILAR COPCORD: World Health OrganizationILAR Community Orientated Program for the Control of Rheumatic
Diseases.
people (95% CI: 130, 352). The most frequent methods for prevalence of SLE to be 177/100 000 (95% CI: 135, 220)
case-finding were local secondary care hospital-based in Afro-Caribbean people and 110/100 000 (95% CI: 58,
outpatient or discharge registries, National Health 163) in West African people compared with 35/100 000
Insurance databases or community surveys, such as the (95% CI: 26, 43) in White European people [103].
World Health OrganizationILAR Community Orientated Studies from the USA have also confirmed the difference
Program for the Control of Rheumatic Diseases (WHO- between Black and White populations [8, 33], with inter-
ILAR COPCORD). mediate figures for Hispanic, Asian and native North
Americans. A study from Hawaii had the greatest ethnic
Age and sex diversity [78]. Here, Chinese and native Hawaiian groups
In all studies, prevalence was highest among females, were most prevalent (24.1 and 20.4/100 000, respectively)
with a female to male ratio ranging between 1.2:1 [86] and Whites least prevalent (5.8/100 000; 95% CI not

and 15:1 [46]. As an example, in Birmingham in the UK, given). In the same study, White people had a significantly
Johnson et al. [29] found estimates of 49.6/100 000 (95% older mean age of disease prevalence of 38.1 years, com-
CI: 43.2, 56.1) for women compared with 3.6/100 000 pared with 29.7 years overall.
(95% CI: 2.0, 6.0) for men in a hospital-based study. A
further study in Birmingham, UK in 1996 aimed to identify Temporal trend
undiagnosed cases of SLE in the community via a postal There appeared to be a trend for increasing prevalence
questionnaire sent to a random sample of 3500 women with time (Fig. 3B). In the UK, the crude annual prevalence
aged 1865 years. This suggested a much greater preva- of SLE reported by Nightingale et al. [76] increased from
lence in women of 200/100 000 (95% CI: 80, 412) [101] 25/100 000 (95% CI: 23.4, 26.7) in 1992 to 40.7/100 000
compared with the hospital-based study. (95% CI: 37.6, 43.8) in 1998. A subsequent study by Rees
Prevalence curves by age had a similar distribution to et al. [1] confirmed this trend and found that prevalence
that of the incidence data, but with a later peak age. rose annually by 3.1% from 1999 to 2012, which was stat-
Figure 2B shows the age- and sex-specific prevalence istically significant. In Malmö, Sweden the prevalence rose
from three papers from selected countries from around from 2.9/100 000 in 1955 to 6.0/100 000 in 1961 [7] and in
the world. Summarizing studies from the UK, the peak Lund and Orup from 39/100 000 (95% CI: 30, 48) on 31
age of prevalence was between 45 and 69 years for fe- December 1982 [70] to 68/100 000 on 31 December 1991
males and between 40 and 89 years for males [1, 76]. [6]. The same trend was found in Northern Norway [11, 20]
Most worldwide studies confirmed the delayed peak age and Minnesota [36, 38].
of incidence in males apart from two studies from
Scandinavia, which found a lower peak age in men [21, Discussion
70].
There are five main findings from this systematic review:
Ethnicity
there is worldwide variation in the reported incidence and
Similar to the incidence data, Black ethnic groups had the prevalence of SLE; in all nationalities, there is a female
highest reported prevalence of SLE, White groups the predominance; there is a peak age of incidence, which
lowest and Asian and Hispanic groups were intermediate occurs in middle-aged adults; Black ethnic groups have
for both males and females. As an example, the preva- the highest incidence and prevalence and White ethnic
lence in different ethnic groups in the UK is summarized in groups have the lowest; and there appears to be an
Table 4. increasing trend in the prevalence of SLE with time.
In addition to the studies in Table 4, a study of women The geographical variation could reflect differences
aged 1564 years in South London estimated the in the genetic mix of populations or variation in
TABLE 4 The prevalence of SLE in the UK by ethnicity
Prevalence per 100 000 (95% CI)
References Region Black Asian White Chinese
Samanta et al. [102] Leicester 40a 20a

Samanta et al. [74] Leicester 64.0a 20.2
Hopkinson et al. [59] Nottingham 207.0 48.8a 20.3a 92.9a
Johnson et al. [29] Birmingham 197.2 96.5a 36.3a
Rees et al. [1] National African: 179.8 Indian: 193.1 134.5 188.39
(125.2, 250.1) (140.8, 258.4) (128.2, 141.1) (90.3, 346.5)
Caribbean: 517.5
(398.5, 660.8)
a
Age-standardized.
Frances Rees et al.
FIG. 3 Temporal trend for the incidence (A) and prevalence (B) of SLE

environmental exposures; for example, countries nearer the study hospitals during the study period, it was not
the equator are exposed to more ultraviolet radiation, an age-adjusted rate, and life expectancy is lower in
which has been hypothesized to be an environmental trig- Zimbabwe such that the peak age of onset may exceed
ger for SLE [104, 105]. The variation could also be attrib- the average life expectancy. Likewise, the low prevalence
utable to differences in the epidemiological study found in Australia may be attributable to the fact that it
methods used, the diagnosis rates of SLE in each country, was a small community survey of Australian Aboriginal
the diagnostic criteria used, access to health care, access people in Yarrabah, North Queensland and was under-
to immunology laboratory tests and differing thresholds powered to detect any SLE cases. The North American
for positive results, the decade the study was carried estimate of SLE incidence of 23.2/100 000 person-years
out, whether the rates were age adjusted and, if not age may be overestimated because it is significantly higher
adjusted, the underlying population structures. For ex- than all the other USA estimates. This may be because
ample, the incidence of SLE in Zimbabwe was one of it is an unadjusted rate or may reflect methodological dif-
the lowest worldwide. This may have been underesti- ferences rather than genetic or environmental differences
mated because the data were collected retrospectively, in the population at risk. This study used the Medicaid
relied on the attendance of people with SLE at one of database, which may have self-selected people with a
chronic disease such as SLE, who may be overrepre- because of improved diagnosis of people with SLE or
sented in Medicaid, and hence increased the estimate. It better case-ascertainment methods in the study design.
should be emphasized that Fig. 1 used data from different Owing to increasing globalization, it is also possibly attrib-
decades and from studies using different case-ascertain- utable to net immigration of non-White populations into
ment methods so should be interpreted with caution. areas that were previously predominantly White. The
In common with other conditions that display autoim- recent reductions in incidence in the UK and the USA
mune features, SLE is universally more common in fe- may therefore reflect changes in environmental risk fac-
males. This could relate both to possession of the tors, such as reduced smoking or changes in migration
double X chromosome and to differences in oestrogen patterns, or perhaps suggest that the risk in later gener-
levels, which modulate immune responses [106, 107]. ations of migrants regresses towards the country’s mean.
Hormonal changes have been hypothesized to explain It is important to study these temporal changes so that

the peak incidence in women in young to middle adult- future health services can be planned to meet the needs
hood compared with childhood and older adulthood. of the populations.
However, this explanation cannot fully explain why the A potential limitation of this study was that, firstly, for
peak in incidence extends into the post-menopausal age completeness, all eligible studies were included regard-
group [2] unless there is a longer latency between the rise less of size or quality. There is therefore a risk of bias
in oestrogen levels, the triggering of the autoimmune affecting the cumulative evidence. In general, earlier stu-
pathway and the development of clinical disease in dies were less rigorous than more recent studies and
some women. there was greater funding of studies in more developed
Incidence and prevalence peak in middle age. Most countries. Secondly, as discussed, it is difficult to assess
worldwide studies confirmed the delayed peak age of trend over time between studies that have used different
prevalence in males. Interestingly, two studies from methodologies. Future work should consider study design
Scandinavia found a lower peak age in men [21, 70]; how- to enable exploration of temporal trends.
ever, this could be attributable to the small numbers of
males in these studies (24 males in the study by Nived et Conclusions
al. [70] and nine males in the study by Lerang et al. [21]).
In summary, there is wide geographical variation in the
The majority of studies that compared ethnic differ-
reported incidence and prevalence of SLE. North
ences found Black people to have high incidence and
America had the highest reported incidence and preva-
prevalence of SLE, White people to have low and
lence of SLE, Africa had the lowest incidence and
Hispanic and Asian people to have intermediate incidence
Australia the lowest prevalence. The incidence and preva-
and prevalence of SLE. However, most of these studies
lence of SLE is higher in females compared with males
were performed in the USA and Europe. Interestingly, the
regardless of age or ethnic origin. The incidence and
study of Black Africans in Zimbabwe [9] had a low inci- prevalence are age related, and there is a peak incidence
dence of SLE. As discussed above, this may have been and prevalence for both sex. Males have an older peak age
underestimated. Alternatively, it may be that the incidence of incidence and prevalence compared with females. In
and prevalence of SLE is higher in Black populations who general, people of Black ethnicity have the highest inci-
have emigrated out of Africa because of differences in dence and prevalence of SLE worldwide, followed by
geneenvironment interactions. This is a hypothesis Asian and then White ethnic groups. There appears to be
being explored in the Gullah population in South a trend of increasing prevalence of SLE with time; the trend
Carolina compared with people from their ancestral for incidence is less clear. Further work to address the lack
origin in Sierra Leone [108, 109]. Further high-quality epi- of epidemiological studies of SLE in Africa, for example
demiological studies in Africa would also help to address using the WHO-ILAR COPCORD approach, may further
this question. This is challenging in a resource-limited knowledge underpinning ethnic variation in SLE.
system, where health-care systems are constrained, but
could be achieved using the approach used by the WHO- Funding: We would like to thank Lupus UK for funding
ILAR COPCORD [110]. towards this project.
It is not possible directly to compare the change in in-
cidence and prevalence between studies in the same Disclosure statement: The authors have declared no
country that have used different study methods or case conflicts of interest.
definitions; for example, in the UK Nightingale et al. [76,
30] used a stricter definition of SLE than Somers et al. [31] Supplementary data
or Rees et al. [1]. The majority of those studies that have
Supplementary data are available at Rheumatology
looked at the same population using the same methods
Online.
over time have shown an increasing incidence and preva-
lence, except for the most recent studies from the UK and
the USA, which showed a reduction in incidence. These
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Rheumatology 2017;56:19451961

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Rheumatology key messages

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TABLE 1 Summary of literature search

Incidence Medline 542 1617 76 46 11

Incidence per 100 000

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Incidence per 100 000

Number of person-years (95% CI)

Uramoto et al. [38] Minnesota Hospital records 48 5.6 (3.9, 7.2)a

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TABLE 4 The prevalence of SLE in the UK by ethnicity

Prevalence per 100 000 (95% CI)

References Region Black Asian White Chinese

Samanta et al. [102] Leicester  40a 20a 

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Rheumatology 2017;56:19451961

Samanta et al. [102] Leicester 40a 20a