Arthritis Care & Research

Vol. 64, No. 2, February 2012, pp 159 –168

DOI 10.1002/acr.20683
© 2012, American College of Rheumatology
ORIGINAL ARTICLE

Systematic Review of the Epidemiology of

Systemic Lupus Erythematosus in the Asia-Pacific
Region: Prevalence, Incidence, Clinical Features,
and Mortality
RUPERT W. JAKES,1 SANG-CHEOL BAE,2 WORAWIT LOUTHRENOO,3 CHI-CHIU MOK,4
SANDRA V. NAVARRA,5 AND NAMHEE KWON1

Objective. Systemic lupus erythematosus (SLE), a chronic multisystem autoimmune disease with a wide spectrum of
manifestations, shows considerable variation across the globe, although there is little evidence to indicate its relative
prevalence in Asia. This review describes its prevalence, severity, and outcome across countries in the Asia-Pacific
region.
Methods. We conducted a systematic literature search using 3 groups of terms (SLE, epidemiology, and Asia-Pacific
countries) of EMBase and PubMed databases and non–English language resources, including Chinese Wanfang, Korean
KMbase, Korean College of Rheumatology, Japana Centra Revuo Medicina, Taiwan National Digital Library of Theses and
Dissertations, and Taiwanese, Thai, and Vietnamese journals.
Results. The review showed considerable variation in SLE burden and survival rates across Asia-Pacific countries.
Overall crude incidence rates (per 100,000 per year) ranged from 0.9 –3.1, while crude prevalence rates ranged from
4.3– 45.3 (per 100,000). Higher rates of renal involvement, one of the main systems involved at death, were observed for
Asians (21– 65% at diagnosis and 40 – 82% over time) than for whites. While infections and active SLE were leading causes
of death, a substantial proportion (6 – 40%) of deaths was due to cardiovascular involvement. The correlation between the
Human Development Index and 5-year survival was 0.83.
Conclusion. This review highlights the need to closely monitor Asian SLE patients in Asian countries for renal and
cardiovascular involvement, especially those who may not receive proper treatment and are therefore at greater risk of
severe disease. We hope this will encourage further research specific to this region and lead to improved clinical
management.

INTRODUCTION tion across the globe, there is little evidence to indicate its
relative prevalence in Asia (1,2). However, clinical mani-
Systemic lupus erythematosus (SLE) is a chronic multisys-
festations of the disease as observed in one study were of
tem autoimmune disease with a wide spectrum of mani-
greater severity in Asia, with greater renal involvement in
festations ranging from minor cutaneous involvement to
particular (3). Probability of long-term survival appears to
severe major organ damage. While the prevalence, sever-
be generally lower in Asia compared to America and Eu-
ity, and outcome of the disease show considerable varia-
rope (4 – 6).

Supported by GlaxoSmithKline.
Dr. Bae’s work was supported by the Korea Healthcare
Technology R&D Project and the Ministry for Health and
Welfare, Republic of Korea (A080588).
1
Rupert W. Jakes, PhD, Namhee Kwon, MD, PhD: Glaxo-
SmithKline, Singapore; 2Sang-Cheol Bae, MD, PhD, MPH:
Hanyang University Hospital for Rheumatic Diseases,
Seoul, South Korea; 3Worawit Louthrenoo, MD: Chiang Mai
University, Chiang Mai, Thailand; 4Chi-Chiu Mok, MD,
FRCP: Tuen Mun Hospital, Hong Kong, China; 5Sandra V.
Navarra, MD: University of Santo Tomas, Manila, Philip-
pines.
Dr. Jakes owns stock in GlaxoSmithKline. Dr. Lou-
threnoo has received consultant fees, speaking fees, and/or
investigator fees (less than $10,000 each) from Roche, Merck
Sharp and Dohme, American Taiwan Biopharm, Actelion,
Pfizer, and TRB Chemedica. Dr. Navarra has received speak-
ing fees (less than $10,000 each) from GlaxoSmithKline,
Roche, and Human Genome Sciences. Dr. Kwon owns stock
in GlaxoSmithKline.
Address correspondence to Rupert W. Jakes, PhD, Glaxo-
SmithKline, 150 Beach Road, #22-00 Gateway West, Singa-
pore 189720. E-mail: rupert.2.jakes@gsk.com.
Submitted for publication December 20, 2010; accepted in
revised form October 17, 2011.

159
160
Significance & Innovations
● This is the first review article that summarizes
published data from English and non–English lan-
guage resources on systemic lupus erythematosus
(SLE) prevalence and incidence as well as the clin-
ical manifestations, long-term outcome, and
causes of mortality among adult SLE patients in
the Asia-Pacific region.
● Higher rates of renal involvement were observed
for Asians (21– 65% at diagnosis and 40 – 82% over
time) than for whites, making it one of the main
organs involved at death. While infections and
active SLE were the leading causes of death, a
substantial proportion (6 – 40%) of deaths was due
to cardiovascular involvement.
● Using the Human Development Index (HDI) as a
proxy for standards of health care and the medical
system, we found a strong correlation (0.83, P ⬍
0.0005) between the HDI and the 5-year survival
rates of SLE patients.
Differences in the causes of mortality have also been
reported among SLE patients in different geographic re-
gions. A bimodal distribution of the cause of death has
been reported in the US, Denmark, and other European
countries (7,8), i.e., mortality late in the course of SLE is
more often due to cardiovascular disease, and mortality
early in the course of SLE is more frequently due to active
disease or infection. In Asia, infections and active SLE
remain the major causes of death both early and late in the
course of the disease (9 –11). However, the typical cohort
followup periods in Asia may be too short to draw conclu-
sions on the latter.
The epidemiology of SLE has been described world-
wide, and there is a suggestion that differences between
Asia and the other parts of the world exist, with Asia
generally showing a poorer prognosis. However, a compre-
hensive review of the available SLE epidemiologic data
in the Asia-Pacific region has not been performed thus far.
This review intends to provide a better understanding of
the patient population in that region and encourage more
efficient clinical management for this population. The ob-
jectives of this review article are the following: 1) to pro-
vide an overview of SLE prevalence and incidence, as well
as the clinical manifestations, long-term outcome, and
causes of mortality among adult SLE patients in the Asia-
Pacific region and 2) to describe any difference in the
disease epidemiology across the countries in the region.
MATERIALS AND METHODS
Literature search. In this review article, the term “Asia-
Pacific” refers to the following countries with available
data: China, Korea, Japan, Taiwan, Hong Kong, Vietnam,
Philippines, Thailand, Malaysia, Singapore, Indonesia,
Australia, and New Zealand. We searched EMBase and
Jakes et al
PubMed from January 1985 to July 2010 and the inclusion
criteria were according to the following search terms: 1)
systemic lupus erythematosus or lupus erythematosus or
lupus or lupus nephritis or CNS lupus; 2) epidemiology or
incidence or prevalence or morbidity or mortality or sur-
vival or severity or hospitalis(z)ation or flare; and 3)
Asia(n) or Australia(n) or Japan(ese) or China or Chinese or
Taiwan(ese) or Hong Kong or Korea(n) or Thai(land) or
Philippines or Filipino(s) or Singapore(an) or Malaysia(n)
or Vietnam(ese) or Indonesia(n) or New Zealand. Addi-
tionally, the search was extended to identify potential
articles from the following non–English language re-
sources: Wanfang Database (Chinese), Korean KMbase,
Korean College of Rheumatology, Japana Centra Revuo
Medicina (Japanese), National Digital Library of Theses
and Dissertations in Taiwan, Index to Taiwan Periodical
Literature System, relevant local Vietnamese journals, and
the local journal of the Thai Rheumatology Association.
Following the identification of articles meeting the objec-
tives of the review, the references were hand searched for
articles missed by the EMBase and PubMed searches.
Study selection. Based on the title and abstract, articles
that met the following criteria were excluded (Figure 1): 1)
commentaries, editorials, conference abstracts, review ar-
ticles, meta-analyses, or studies undertaken in countries
outside those prespecified; 2) animal, autopsy, genetic,
laboratory, pathology, or renal biopsy studies; 3) therapeu-
tic intervention studies, quality of life, or disease cost
studies; 4) case reports or case series; 5) selected patient
groups (pregnancy, pediatric, inpatients, patients with
specific comorbidity, lupus nephritis); 6) association stud-
ies (e.g., smoking and risk of SLE); and 7) studies of the
validation of questionnaires. Full manuscripts of the re-
sulting articles were retrieved and carefully reviewed for
inclusion by applying the above criteria; 48 articles were
excluded as a result. In addition, 1 study was excluded as
it based the definition of SLE upon insurance claims codes
rather than standard criteria, 7 studies were excluded as
the population described was a subset of those reported in
selected articles, and 7 studies where there was no rele-
vant data to extract were also excluded.
Our literature search yielded 3,650 articles from EMBase
and 2,341 articles from PubMed. After reviewing these
articles and applying the above exclusion criteria, 35 arti-
cles remained that met our criteria for inclusion. In addi-
tion, 1 article from the non–English language search and 2
articles found from hand searching references from se-
lected articles were included. Therefore, this review is
based on 38 articles that fulfilled our criteria and reported
data on prevalence, incidence, clinical manifestations,
survival, or causes of mortality among adult SLE patients
in the countries of interest. Of the 38 selected studies, 3
were conducted in China, 4 in Korea, 4 in Japan, 2 in
Taiwan, 6 in Hong Kong, 1 in Vietnam, 4 in the Philip-
pines, 2 in Thailand, 4 in Malaysia, 4 in Singapore, 3 in
Australia, and 1 in New Zealand.
Data extraction. All selected articles included patients
who fulfilled at least 4 of the American College of Rheu-
matology (ACR) criteria for SLE (12,13). We extracted data
SLE in the Asia-Pacific Region
Figure 1. Study selection flow chart. SLE ⫽ systemic lupus erythematosus.
on prevalence, incidence, demographic profile, clinical
manifestations, laboratory features, survival, and causes of
death from the selected articles where available and orga-
nized them into tables or graphs, which were sorted by
countries according to their geographic location and or-
dered from north to south. Where data in an article in-
cluded patients with childhood onset of SLE (14 –16), we
excluded these patients during the data extraction.
We reported incidence as the number of new cases per
100,000 of population per year and prevalence as the num-
ber of cases per 100,000. In articles where only sex-specific
incidence or prevalence rates were provided, we calcu-
lated the overall rate as an average of the male and female
rates. In articles where results were stratified, data on
161
demographic profile, clinical manifestations, laboratory
features, or causes of death were combined and summa-
rized as weighted mean, median, or percentage accord-
ingly. Data on demographic profile, clinical manifesta-
tions, or laboratory features, if available from more than 1
article for the same country, were combined and summa-
rized accordingly.
Human Development Index (HDI) analysis. The HDI is
a composite statistic that can be used to rank countries in
terms of their economic and social development; its com-
ponents include national income, educational attainment,
and life expectancy. Using the HDI as a proxy for standards
162 Jakes et al

Table 1. Incidence and prevalence of SLE in the Asia-Pacific region*

Incidence Prevalence
(per 100,000 per year) (per 100,000)

Cases,
Source, ref. Country Period no.† Ethnicity Overall Female Male Overall Female Male

Iseki et al, 17Japan, Okinawa 1973–1991 35–466 NS 0.9–2.8‡ 1.4–4.7 0.4–0.8 4.3–37.7‡ 7.7–68.4 0.8–7.0
Kameda, 18 Japan, 1975–1977 108 NS 1.03 ND ND 10.8 ND ND
Fukuoka City
Mok et al, 19 Hong Kong 2000–2006 272–442 Chinese 3.1 5.4 0.8‡ ND ND ND
Bossingham, 20 Australia, 1998 108 All§ ND ND ND 45.3 ND ND
Queensland
26 Aborigines ND ND ND 92.8 ND ND
Segosothy and Australia, 1996–1997 14 Aborigines ND ND ND 73.5 ND ND
Phillips, 21 Central
6 Whites ND ND ND 19.3 ND ND
Anstey et al, 22 Australia, 1986–1990 13¶ Aborigines 11 ND ND 52.6 ND ND
Northern
Territory

* Crude rates, not age adjusted. SLE ⫽ systemic lupus erythematosus; NS ⫽ not specified; ND ⫽ not done.
† Prevalent cases if not otherwise indicated.
‡ Derived from reported estimates. Where only sex-specific incidence or prevalence rates were provided, the overall rate was calculated as an average
between male and female rates.
§ Includes 78 patients of European or mixed descent (72%), 26 Aborigines (24%), 2 Sikhs (2%), 1 Filipino (0.9%), and 1 Indonesian (0.9%).
¶ Incident cases.

of health care and the medical system, we calculated the
correlation coefficient for the association between the HDI
and 5-year survival rates of the studies identified in our
systematic review. The HDI values for the calendar year
corresponding to the year of survival estimates were used
(http://hdr.undp.org/en/data/explorer).
RESULTS
Incidence and prevalence in frequency of SLE. The in-
cidence and prevalence of SLE are summarized in Table 1.
Overall crude incidence rates (per 100,000 per year)
ranged from 0.9 –3.1, while crude prevalence rates ranged
from 4.3– 45.3 (per 100,000) in the cited Asia-Pacific coun-
tries, with a trend toward higher prevalence of SLE in
Australia compared to the cited Asian countries (45.3 ver-
sus 4.3–37.7). SLE was more common in Aborigines (11 for
incidence and 52.6 –92.8 for prevalence) compared to
whites (19.3 for prevalence) in Australia and Asians in the
cited Asia-Pacific countries who were mainly of Chinese
(3.1 for incidence) or Japanese (0.9 –2.8 for incidence and
4.3–37.7 for prevalence) ethnicity. It also affected women
much more frequently than men (1.4 –5.4 versus 0.4 – 0.8
for incidence and 7.7– 68.4 versus 0.8 –7.0 for prevalence).
countries. SLE duration (since diagnosis of SLE) ranged
from 1.4 –9.3 years, with longer disease duration observed
among patients in Australia (7.6 years) and Hong Kong (9.3
years).
The main clinical manifestations and laboratory find-
ings at diagnosis and during the course of SLE of the adult
patients described in Table 2 are illustrated in Figure 2.
Nonerosive arthritis, malar rash, and renal disorder were
the most common clinical manifestations observed at di-
agnosis (ranging from 31– 80%, 48 – 63%, and 14 – 65%,
respectively) and over time (ranging from 48 – 83%, 41–
84%, and 30 – 82%, respectively). The most frequently
observed laboratory findings at diagnosis and over the
course of the disease were positive antinuclear antibodies,
ranging from 93–100% and 55–100%, respectively, anti-
DNA ranging from 51– 86% and 41– 83%, respectively,
and hematologic disorder ranging from 19 – 61% and 67–
87%, respectively.
The clinical and laboratory profile appeared broadly
similar across the populations in the Asia-Pacific region
except for strikingly lower rates of renal involvement ob-
served in New Zealand (14% at diagnosis) and Australia
(30% over time) compared to the Asian countries (21– 65%
at diagnosis and 40 – 82% over time).

Demographic and clinical features of SLE. The demo-
graphic profile of adult SLE patients in the Asia-Pacific
region is summarized in Table 2. A preponderance of
female patients was consistently observed across the cited
countries, ranging from 83–97% (excluding studies that
recruited only female or male patients). The mean age at
SLE onset ranged from 25.7–34.5 years, with patients in
Malaysia (25.7 years) and Philippines (26.7 years) demon-
strating earlier onset compared to patients in the other
Survival and main causes of death in SLE. Survival
rates in the Asia-Pacific region are summarized in Table 3.
Survival rates ranged from 93–98% at 1 year, 60 –97% at 5
years, and 70 –94% at 10 years (excluding survival data
specified as calculated from SLE onset) across the cited
countries, with a general trend of improvement in survival
for more recent studies. Among the cited countries in the
Asia-Pacific region, prognosis of SLE appeared to be better
in China (Shanghai; 98% at 5 years), Hong Kong (97% at 5
SLE in the Asia-Pacific Region 163

Table 2. Demographic profile of adult SLE patients in the Asia-Pacific region*

Age at SLE Age at SLE

Country, Study Cases, Age, Female, onset, diagnosis, Duration of SLE,
ref. period† no. years‡ % years‡ years‡ years‡

China 31.7 30.9 ⫾ 9.6 NS 5.3 ⫾ 3.9

24 2010§ 1,682 68
23 1959–1993 566 90
Korea 32.3 ⫾ 11.3 28.9 ⫾ 10.9 NS 4.1 (0.5–22.3)
25 1997–2007 914 100¶
26 1992–2005 588 94
Japan NS NS NS NS
27 1975–2004 306 90
18 1975–1977 108 95
Taiwan NS 34.5 ⫾ 11.6 34.9 ⫾ 11.3 3.2 ⫾ 3.0
28 1988–1998 194 83
29 1983–1996 72 0¶
Hong Kong 41 ⫾ 11 29.8 ⫾ 12.8 33.1 ⫾ 12.1 9.3 ⫾ 6.6
32 2007 1,082 90
30 2006–2007 306 96
19 2000–2006 442 91
15 1991–2003 235 92
33 1982–1991 137 94
31 1985–1989 156 96
Vietnam
34 1996–2000 438 NS 94 NS NS NS
Philippines 26.7 28.5 ⫾ 11.5 1.4
35 2005 1,070 NS 96
36 1993§ 75 NS 97
Thailand 32.1 ⫾ 11.7 30.1 31.6 ⫾ 10.7 2.0 (0.02–16.3)
37 1986–2000 349 97
16 1990–1992 335 90
Malaysia 32.1 25.7 ⫾ 10 27 ⫾ 10 2.5 ⫾ 3.9
38 1999–2000 82 89
39 1997–1998 134 91
40 1976–1990 494 93
Singapore NS 29.6 (13–63) 31.2 (16.0–75.0) 5.48 (0.1–30.6)
3 1995–1996 472 92
41 1980–1992 175 94
42 1988§ 94 91
Australia NS 32.9 31.1 7.6
21 1990–1999 24 83
20 1996–1998 108 86
22 1984–1991 22 95
New Zealand NS NS 34.1 ⫾ 13.8 NS
43 2000–2005 170 93

* SLE ⫽ systemic lupus erythematosus; NS ⫽ not specified.

† Studies were sorted according to the last year of the study period.
‡ Values are the mean ⫾ SD or the median (range).
§ Indicates publication year.
¶ Study selected only female or male patients.

years and 94% at 10 years), and South Korea (94% at 5
years), while the Aborigines in Australia showed poor
prognosis at 5 years (60%).
The main causes of death in SLE in the Asia-Pacific
region are summarized in Table 4. Infections (30 – 80%)
and active SLE disease (19 –95%) were the leading causes
of death among the cited countries in the Asia-Pacific
region, and cardiovascular (6 – 40%) and renal involve-
ment (7–36%) were the main systems involved at death.
Analysis of HDI. For the 13 studies where 5-year sur-
vival rates were available, the correlation between the HDI
and 5-year survival was 0.83 (P ⬍ 0.0005). We excluded
the Australian study (22) as its subjects were rural Aborig-
ines, and we felt that the very high HDI for Australia did
not represent the Aborigine population.
DISCUSSION
This is the first review article that summarizes published
data from English and non–English language resources on
SLE prevalence and incidence, as well as the clinical man-
ifestations, long-term outcome, and causes of mortality
164 Jakes et al

Figure 2. Frequency of key clinical features at diagnosis (A), cumulative frequency of clinical features during disease course (B), frequency
of laboratory features at diagnosis (C), and cumulative frequency of laboratory features during the disease course (D) of systemic lupus
erythematosus in the Asia-Pacific region. ACA ⫽ anticardiolipin antibodies; LAC ⫽ lupus anticoagulant; STS ⫽ serologic test for syphilis;
ANA ⫽ antinuclear antibodies.

among adult SLE patients in the Asia-Pacific region. It also the cited countries in the Asia-Pacific region. Most of our
describes differences in the disease epidemiology ob- cited studies from Australia showed that Aborigines have
served across the cited countries in that region. higher SLE rates than local whites. While these studies
We found considerable variation in SLE burden across concurred with an earlier review of white populations in
SLE in the Asia-Pacific Region 165

Table 3. SLE survival rates in the Asia-Pacific region*

Survival rates (%)

Study Cases,
Source, ref. Country period no. Ethnicity 1-year 5-year 10-year

Chen et al, 9 China, Shanghai 1980–1998 50 NS 98† 98† 84†

98‡ 86‡ 76‡
Xie et al, 23 China, Shanghai 1959–1993 566 NS 93† 73† 60†
Kim et al, 44 Korea 1993–1997 544 Korean 98 94 ND
Iseki et al, 17 Japan, Okinawa 1972–1993 566 NS 97‡ 89‡ 78‡
Kameda, 18 Japan, 1975–1977 103 NS ND 89 64
Fukuoka City (females)
Funauchi et al, 27 Japan, Osaka 1975–2004 306 NS ND ND 89
Pu et al, 28 Taiwan 1988–1998 152 NS, onset age ⬍50 years 97 88 82
21 NS, onset age 50–64 years 76 66 54
21 NS, onset age ⱖ65 years 74 55 55
Chang et al, 29 Taiwan 1983–1996 72 Chinese, male 85‡ 76‡ 75‡
519 Chinese, female 89‡ 81‡ 78‡
Mok et al, 15 Hong Kong 1991–2003 213 Chinese, adult onset§ ND 94¶ 87¶
22 Chinese, late onset§ ND 66‡ 44‡
Wong, 31 Hong Kong 1985–1989 156 Chinese ND 97 94
Amante, 36 Philippines 1993# 75 Filipinos 93** 88** ND
Victorio et al, 45 Philippines 1982–1988 102 Filipinos 94‡ 75‡ ND
Kasitanon et al, 37 Thailand 1986–2000 349 Thai ND 84 75
Paton et al, 11 Malaysia 1978–1993 102 All (Chinese 47%, Malays 93 86 70
46%, Indians 7%)
Anstey et al, 22 Australia, 1984–1991 22 Aborigines 91 60 ND
Northern
Territory

* Time zero for calculation of survival rates were not specified unless otherwise stated. SLE ⫽ systemic lupus erythematosus; NS ⫽ not specified; ND ⫽
not done.
† From SLE onset.
‡ From SLE diagnosis.
§ Adult onset ⫽ age 16 –50 years; late onset ⫽ age ⬎50 years.
¶ From SLE diagnosis; exact survival rates were not indicated in the cited article. Rates were extrapolated from the survival curve shown in the article.
# Indicates publication year.
** From SLE diagnosis; derived from reported cumulative mortality rate.

the US, Europe, Canada, and Australia, as reported by
Danchenko et al (1), our findings do not provide sufficient
evidence to conclude whether SLE is more common in
some countries or races in the Asia-Pacific region com-
pared to others due to limited epidemiologic data from
studies in this region. Further, it should be noted that
while disease burden may be influenced by true differ-
ences across the countries or races, other differences be-
tween studies, such as year of study and whether inci-
dence of a single year or over a period of time was
reported, and methodologic issues such as study design,
referral pattern, inconsistencies in definition of ACR cri-
teria for SLE, and method of case ascertainment also con-
tribute to variability in the reported rates.
It has been reported that age at SLE onset or diagnosis is
higher among white populations (38 –51.7 years) com-
pared to nonwhite populations (20 –32.3 years for Asian
populations and 32.2 years for Aborigines) (48). Our re-
view showed that the mean age at SLE onset (25.7–34.5
years) or diagnosis (27–34.9 years) in the cited Asia-Pacific
countries was similar to that reported in nonwhite popu-
lations. This could be attributed to the high proportion of
nonwhite populations in the cited studies from Australia
(28 –100% Aborigines) and New Zealand (59%) (20 –22,43).
Our review showed that frequencies of renal involve-
ment at diagnosis and over the course of SLE in the cited
Asia-Pacific countries ranged from 14 – 65% and 30 – 82%,
respectively, with higher rates of renal involvement ob-
served among the cited Asian countries (21– 65% at diag-
nosis and 40 – 82% over time) compared to New Zealand
(14% at diagnosis) and Australia (30% over time). This
concurs with reports of renal involvement being more
common among Asian populations than white popula-
tions (2) and highlights the need to closely monitor the
Asian populations in the Asia-Pacific region who may not
be treated properly and are therefore at greater risk of
severe disease. None of the selected studies specifically
studied comorbidities. Therefore, available data were
scarce and did not present a good picture of the comor-
bidities related to the disease in the Asia-Pacific region.
There was considerable variation in survival rates across
the cited countries in the Asia-Pacific region. The 5-year
survival rates ranged from 60% among the Aborigines in
Australia to 94% in South Korea, 97% in Hong Kong, and
98% in China (Shanghai), while 10-year survival rates
ranged from 70% in Malaysia to 94% in Hong Kong. Dif-
ference in survival might result from a delay in treatment
and poor compliance of patients. These may vary across
countries because of cultural differences, medical systems,
educational background, and socioeconomic factors. Other
166 Jakes et al

Table 4. Underlying causes of death in SLE in the Asia-Pacific region*

Country, Age at Duration

no. of death, of SLE, Ethnicity
Source, ref. deaths years years (%) Underlying causes of death (%)†

Chen et al, 9 China, 16 35.6 ⫾ 13.1 11.4 ⫾ 5.2 NS Infection (31), renal (31), cardiovascular (13),
cerebrovascular (13), GI vasculitis (6),
unknown (6)
Kim et al, 44 Korea, 40 33.8 ⫾ 13.6 3.9 ⫾ 1.8 Korean Infection (33), active SLE (25), cardiovascular
(18), cerebrovascular (10), hematologic (8),
pulmonary (3), GI (3), unknown (3)
Chun and Bae, Korea, 10 NS NS Korean Active SLE (80), infection (10), suicide (10)
46
Ichikawa et al, Japan, 212 33.3 ⫾ 11.3 NS NS Infection (35), active SLE (27), cardiovascular (7),
47 cerebrovascular (10), GI (7), suicide (6),
malignancy (3), hepatic (2), others (2),
unknown (1)
Pu et al, 28 Taiwan, 36 NS NS Chinese Infection (69), renal (17), pulmonary (14),
cerebrovascular (6), CNS (6), malignancy (6),
unknown (8)‡
Chang et al, 29 Taiwan, 15 NS NS Chinese Infection (33), renal (20), CNS (20), pulmonary
(13), cerebrovascular (7), malignancy (7)
Mok et al, 19 Hong Kong, 43.8 ⫾ 17.4 5.1 ⫾ 5.9 Chinese Infection (60), cerebrovascular (10), renal (7),
30 cardiovascular (6), malignancy (3), suicide (3),
unknown (10)
Wong, 31 Hong Kong, NS NS Chinese Active SLE (60), infection (40)
5
Lee et al, 33 Hong Kong, NS NS Chinese Cerebrovascular (45), renal (36), infection (23),
137 cardiovascular (9), suicide (5), GI (5)
Amante, 36 Philippines, NS NS Filipinos Infection (50), infection and active SLE (30),
10 active SLE (10), cardiovascular and renal (10),
pancreatitis (10)‡
Lanzon and Philippines, NS NS Filipinos Active SLE (56), infection (35), cardiovascular
Navarra, 14 200 (16), cerebrovascular (10), malignancy (1),
suicide (1)
Kasitanon Thailand, NS NS Thai Infection (52), active SLE (35), cardiovascular (4),
et al, 37 52 thromboembolism (2), malignancy (2),
transfusion reaction (2), iatrogenic
intraabdominal hemorrhage (2), unknown (2)
Paton et al, 11 Malaysia, 28 3.3 NS Infection (52), active SLE (19), cerebrovascular
21 (14), thromboembolism (5), GI and renal (5),
unknown (5)
Yeap et al, 40 Malaysia, 28.6 ⫾ 11.1 1.0 (0.1–14.1) Chinese (73) Infection (30), renal (15), pulmonary (14),
100 Malays (18) cardiovascular (7), CNS (5), malignancy (1),
Indians (9) acuteanaphylaxis (1), unknown (27), SLE as a
contributory factor of death (19)‡
Koh et al, 10 Singapore, 35.1 ⫾ 14 4.0 (0.1–20.8) Chinese (79) Active SLE (45), infection (40), thromboembolism
67 Malays (16) (8), malignancy (6), cardiovascular (2)
Indians (9)
Others (2)
Bossingham, Australia, 9 NS 9.2 Aborigines (67) Thromboembolism (56), active SLE or treatment
20 European (22) complications (33), suspected
Sikh (11) thromboembolism (11)
Segasothy and Australia, 2 36 1.2 Aborigines Infection (50), thromboembolism (50)
Phillips, 21
Anstey et al, Australia, 9 30.0 2.9 Aborigines Infection (67), cardiovascular (22), renal (11)
22

* Age and duration are shown as the mean ⫾ SD or median (range) unless otherwise specified. SLE ⫽ systemic lupus erythematosus; NS ⫽ not
specified; GI ⫽ gastrointestinal; CNS ⫽ central nervous system.
† CNS includes lupus, CNS involvement, and neurologic disease. Pulmonary includes pulmonary disease, respiratory disease, interstitial lung disease,
pulmonary hemorrhage syndrome, pulmonary hemorrhage, acute respiratory distress syndrome, pulmonary edema, and pneumonitis. Cardiovascular
includes pulmonary hypertension, rheumatic heart disease, myocarditis, cardiac death, and ruptured aortic aneurysm. Pulmonary embolism includes
thromboembolism.
‡ Percentages may not add up to 100% due to rounding or subjects in cited reference may have more than 1 cause of death.
SLE in the Asia-Pacific Region
reasons for this observation include differences in study
methodology (such as those mentioned above) and other
factors such as nonstandardization of “time zero” for cal-
culation of survival rates.
Vasudevan and Krishnamurthy (49) undertook an ana-
lysis to describe the HDI against survival of SLE patients at
5 years and found a correlation of 0.56. Using the HDI as a
proxy for standards of health care and the medical system,
we calculated the correlation coefficient by plotting the
HDI against 5-year survival rates of the studies identified
in our systematic review. For the 13 studies where 5-year
survival rates were available, we found a strong correlation
(0.83, P ⬍ 0.0005). As countries achieve higher economic
and social status, we might see improved outcome and
survival in SLE patients.
We found, similar to reports on causes of death among
SLE patients in Asia (50), that infections and active SLE
disease remain the leading causes of death. The second
peak of death due to cardiovascular disease was not ob-
served as in the Western countries. This might be due to
the short duration of followup in most cited articles. Renal
involvement remains as one of the main organ systems
involved at death among the SLE populations in the Asia-
Pacific region. Notably, however, there appeared to be a
substantial proportion of deaths due to cardiovascular in-
volvement (6 – 40%) in the Asia-Pacific region. This high-
lights the importance of increasing the awareness of the
risks of cardiovascular death for SLE patients whose sus-
ceptibility to the disease increases as they age and to
implementing proactive measures to reduce the risk fac-
tors for cardiovascular disease in this population.
We recognize that regional differences in SLE epidemi-
ology observed across the countries may be related to
factors such as differences in environment, age at onset,
sex, socioeconomic status, availability and accessibility to
health care resources, availability of therapy, compliance
to medical care, and methodologic issues across the stud-
ies. Some of these factors need to be considered when
making comparisons across studies. Nonetheless, this ar-
ticle provides valuable information on the burden of SLE,
in particular prevalence and incidence, as well as the
clinical manifestations, long-term outcome, and causes of
mortality among adult SLE in the Asia-Pacific region. This
may encourage further research on SLE to address issues
specific to this region, which may eventually lead to more
efficient clinical management of this population.
ACKNOWLEDGMENTS
Medical writing support was provided by Hui-Hwa Choo
and Sen-Kwan Tay.
AUTHOR CONTRIBUTIONS
All authors were involved in drafting the article or revising it
critically for important intellectual content, and all authors ap-
proved the final version to be submitted for publication. Dr. Jakes
had full access to all of the data in the study and takes responsi-
bility for the integrity of the data and the accuracy of the data
analysis.
Study conception and design. Jakes, Bae, Louthrenoo, Mok, Na-
varra, Kwon.
167
Acquisition of data. Jakes, Bae, Louthrenoo, Mok, Navarra, Kwon.
Analysis and interpretation of data. Jakes, Bae, Louthrenoo, Mok,
Kwon.
ROLE OF THE STUDY SPONSOR
GlaxoSmithKline oversaw the study design, data collection,
data analysis, and writing of the manuscript, and approved the
content of the submitted manuscript. Publication of this article
was not contingent on the approval of GlaxoSmithKline.
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