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Epidemiology and survival of systemic lupus erythematosus in Hong Kong Chinese

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cc Mok at Tuen Mun Hospital
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Systemic lupus erythematosus (SLE) is a fairly common rheumatic disease in Hong Kong, China. The prevalence and annual incidence of SLE are estimated to be 0.1% and 6.7/100,000 population, respectively. The 10-year cumulative survival of SLE patients in Hong Kong is 83% and the age and gender-adjusted standardized mortality ratio was 5.25 (1.64–10.4) from 1999 to 2008. The commonest cause of death is infections (60%), followed by cardiovascular complications (16%). Life expectancy analysis reveals a loss of 20 years in women and 27 years in men when SLE develops at birth. The loss in life years is greatest in the younger age groups. Renal damage is the most frequent disease-related damage, whereas musculoskeletal damage is the commonest treatment-related complication. The quality of life of our SLE patients is impaired and declines over time, which is contributed by new organ damage. One-third of our patients lose their ability to work within 5 years of disease onset, which is mainly attributed to musculoskeletal pain, fatigue, anxiety and depression symptoms, and memory deterioration. With the availability of novel therapeutics and an increased awareness of complication prevention in SLE, it is expected that our patients will live longer with a better quality of life in the next decade.
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Lupus (2011) 20, 767–771
http://lup.sagepub.com
LUPUS AROUND THE WORLD
Epidemiology and survival of systemic lupus
erythematosus in Hong Kong Chinese
CC Mok
Department of Medicine, Tuen Mun Hospital, Hong Kong, China
Systemic lupus erythematosus (SLE) is a fairly common rheumatic disease in Hong Kong,
China. The prevalence and annual incidence of SLE are estimated to be 0.1% and 6.7/100,000
population, respectively. The 10-year cumulative survival of SLE patients in Hong Kong is
83% and the age and gender-adjusted standardized mortality ratio was 5.25 (1.64–10.4) from
1999 to 2008. The commonest cause of death is infections (60%), followed by cardiovascular
complications (16%). Life expectancy analysis reveals a loss of 20 years in women and 27 years
in men when SLE develops at birth. The loss in life years is greatest in the younger age groups.
Renal damage is the most frequent disease-related damage, whereas musculoskeletal damage
is the commonest treatment-related complication. The quality of life of our SLE patients is
impaired and declines over time, which is contributed by new organ damage. One-third of our
patients lose their ability to work within 5 years of disease onset, which is mainly attributed to
musculoskeletal pain, fatigue, anxiety and depression symptoms, and memory deterioration.
With the availability of novel therapeutics and an increased awareness of complication pre-
vention in SLE, it is expected that our patients will live longer with a better quality of life in
the next decade. Lupus (2011) 20, 767–771.
Key words: Chinese; complications; life expectancy; morbidity; mortality; survival
Introduction
Systemic lupus erythematosus (SLE) is a prototype
autoimmune disease that predominantly affects
women of childbearing age. SLE is fairly common
in Asian countries, including mainland China and
Hong Kong. Epidemiological studies of SLE are
important for assisting physicians to understand
the impact and outcome of this devastating
and potentially lethal disease in the community.
Information on the incidence, prevalence, mortality
and various comorbidities of SLE, and their trend
over time, is valuable for health care administrators
to plan for medical services to meet the population
need and improvement in the quality of care to
patients suffering from the disease.
To understand the frequency of the disease in the
population, its trend over time, clinical patterns of
manifestations, morbidities, survival and various
other disease outcomes such as organ complica-
tions, quality of life and disability, clinical registries
and different assessment instruments are manda-
tory. Hong Kong is a small city situated in the
southern part of China and has a population of
7 million in the year 2010. All Hong Kong citizens
are entitled to receive medical care through a pub-
lic medical system heavily subsidized by the
Government. Most Hong Kong citizens utilize
public medical health care, particularly for more
serious or chronic illnesses such as rheumatic dis-
eases. Currently, there are 37 public hospitals in
Hong Kong, which are under the jurisdiction of
the Hospital Authority. Since more than a decade
ago, a computer system has been established in all
Hospital Authority hospitals and outpatient clinics
for electronic recording of medical consultations
and data capture. This registry and information
retrieval system, called the Clinical Data Analysis
and Reporting System (CDARS), captures diag-
nostic codes according to the International
Classification of Disease, mortality and causes of
deaths, headcounts of various disease entities, sur-
gical procedures and different kinds of morbidities.
This system is thus a powerful tool to facilitate
Correspondence to: Chi Chiu Mok, Department of Medicine, Tuen
Mun Hospital, Tsing Chung Koon Road, New Territories,
Hong Kong, SAR China
Email: ccmok2005@yahoo.com
Received 22 August 2010; accepted 27 September 2010
!The Author(s), 2010. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203310388447
... Similarly, the clinical significance of low C3 and C4 circulating levels as biomarkers for LN is still matter of research [29]. Whereas a significant drop in C4 levels can be observed even two months prior to renal flare occurrence, a decline in C3 was shown to be influenced by genetic variants of factor H, which regulates C3-convertase in the alternative pathway [30]. In addition, elevated titers of autoantibodies directed against C1q have been described as better predictors of renal involvement in SLE patients compared to C3 and C4, although with inconclusive results [30]. ...
... Whereas a significant drop in C4 levels can be observed even two months prior to renal flare occurrence, a decline in C3 was shown to be influenced by genetic variants of factor H, which regulates C3-convertase in the alternative pathway [30]. In addition, elevated titers of autoantibodies directed against C1q have been described as better predictors of renal involvement in SLE patients compared to C3 and C4, although with inconclusive results [30]. Further analysis of our data revealed significantly lower levels of C3 and C4 in pregnant patients with flare at all time-points considered, from conception throughout pregnancy and until 3 months following delivery, consistently with what reported by other authors [30]. ...
... In addition, elevated titers of autoantibodies directed against C1q have been described as better predictors of renal involvement in SLE patients compared to C3 and C4, although with inconclusive results [30]. Further analysis of our data revealed significantly lower levels of C3 and C4 in pregnant patients with flare at all time-points considered, from conception throughout pregnancy and until 3 months following delivery, consistently with what reported by other authors [30]. Most importantly, the present study also highlights that those patients with both previous LN and disease flare during gestation had the lowest complement levels, suggesting that levels of C3 and C4 below the normal threshold before conception can serve as predictor of flare during pregnancy in this high-risk group of patients [31]. ...
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... The prevalence of SLE in mainland China is about 30-70/100,000, and the ratio of males to females is 1:10-12 [3]. It is estimated that across all Hospital Authority (HA) hospitals in Hong Kong, there were 5243 patients with SLE in 2008, 91% of whom were women [4]. The prevalence of SLE in Hong Kong was calculated to be 0.1% [4] Therapyor disease-related comorbidities may occur in SLE patients. ...
... It is estimated that across all Hospital Authority (HA) hospitals in Hong Kong, there were 5243 patients with SLE in 2008, 91% of whom were women [4]. The prevalence of SLE in Hong Kong was calculated to be 0.1% [4] Therapyor disease-related comorbidities may occur in SLE patients. A study showed a high risk of comorbidity at the time of diagnosis and incident comorbidity after diagnosis in patients with SLE [5]. ...
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... wider: 20% in Malaysia, 38 16% in the Euro-Lupus cohort, 39 22.1% in the Latin American Grupo Latino Americano De Estudio del Lupus (GLADEL) cohort, 40 16.4% in the Chinese CSTAR cohort, 41 32.2% in Egypt 42 43 and 19.9% in Hong Kong. 44 The wide range in Saudi research differs from the stable rates seen in these global groups. Saudi patients with SLE exhibit different rates of cardiopulmonary symptoms compared with patients with SLE globally. ...
Systematic review of the reporting of extrarenal manifestations in observational studies of Saudi patients with systemic lupus erythematosus
Article
Full-text available
  • Mar 2025
Background SLE is prevalent in Saudi Arabia, with numerous studies focusing on SLE in adult patients. However, there is a lack of comprehensive studies summarising the extrarenal manifestations of SLE in this population. This study aims to assess the variability in the prevalence rates of extrarenal manifestations of SLE across different cities in Saudi Arabia and to emphasise the need for a national registry to better understand the overall disease burden in the region. Methods We conducted a systematic review of articles with no time restrictions, including studies from databases such as Medline, ScienceDirect, EBSCO and PubMed up to July 2024. The review process involved screening, data extraction and quality assessment in duplicate. Only observational or experimental studies focusing on extrarenal manifestations in adult patients with SLE in Saudi Arabia were included. The review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) checklist for systematic reviews to ensure a rigorous and comprehensive evaluation. Results A total of 35 studies were included, primarily retrospective cohort studies. Riyadh showed the highest number of publications over time. Musculoskeletal involvement in SLE ranged from 2% to 100%, with most studies reporting 46%–85%. Mucocutaneous manifestations, including discoid rash (5%–100%), malar rash (up to 79%) and photosensitivity (6.12%–29.3%), varied widely. Raynaud’s phenomenon was noted at 4.5%–15.2%. Constitutional symptoms were more common in early-onset SLE, while serositis and cardiopulmonary issues showed variability. Neuropsychiatric symptoms, especially depression, reached up to 67.6%. Conclusion This study explores the prevalence of extrarenal manifestations of SLE among adult Saudi patients, highlighting significant regional variability in musculoskeletal, dermatological, cardiovascular and neurological symptoms. It addresses a gap in the literature for a region where autoimmune diseases are a growing public health concern. The findings emphasise the need for population-based studies to investigate environmental, genetic and lifestyle factors influencing SLE progression.
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... 1 2 SLE can affect patients' ability to work, and they might lose their jobs within 5 years of diagnosis. 2 The prevalence and incidence of SLE varies across different regions and populations. ...
Effect of Benson relaxation response technique on the quality of life among patients with systemic lupus erythematous: quasi-experimental study
Article
Full-text available
  • Jan 2025
Objectives To investigate the effect of Benson relaxation response technique (BRRT) on the quality of life (QOL) among patients with systemic lupus erythematous (SLE). Methodology A quasi-experimental design was used to conveniently recruit 170 patients with SLE. Participants were divided into two groups, the control and the intervention group for which the BRRT intervention was administered. Utilising an online questionnaire, the QOL was assessed among the two groups, before and 2 months after the intervention, using the Arabic version of the short form 36-item health survey. Results After 2 months of the intervention, the intervention group exhibited significantly higher levels in both components of QOL; physical ( t (143.31)=15.35, p<0.001); and mental component ( t (143.58)=12.35, p<0.001). Additionally, for the intervention group, the results revealed a statistically significant increase in the levels of both components from baseline measurement; physical ( t (84)=−16.24, p<0.001) and mental component ( t (84)=−12.93, p<0.001). Conclusion The findings demonstrate a notable positive impact of BRRT on QOL among patients with SLE. Healthcare professionals can potentially improve the overall well-being of patients with SLE and complement traditional treatment by implementing BRRT into their care.
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... [6][7][8] The estimated prevalence rate of the Chinese population is approximately 50-100/100 000. 9 In addition, China has a large population, and SLE has become an important disease burden affecting the health of the Chinese people. An important part of SLE pathogenesis is production of autoantibodies. ...
Abnormally high expression of D1-like dopamine receptors on lupus CD4+ T cells promotes Tfh cell differentiation
Article
Full-text available
  • Aug 2023
Objective: SLE is a chronic autoimmune disease that places a great burden on human society. T follicular helper (Tfh) cells play a critical role in the pathological process of SLE. Therefore, elucidating the mechanism of Tfh cell differentiation will contribute to SLE treatment. Dopamine receptors (DRDs) are members of the family of G protein-coupled receptors and are primarily divided into D1-like and D2-like receptors. Previous studies have found that DRDs can regulate differentiation of immune cells. However, there is currently a lack of research on DRDs and Tfh cells. We here explore the relationship between DRDs and Tfh cells, and analyse the relationship between DRD expression on Tfh cells and the course of SLE. Methods: We first detected plasma catecholamine concentrations in patients with SLE and healthy controls by mass spectrometry, followed by reverse transcription-quantitative PCR (RT-qPCR) to detect DRD messenger RNA (mRNA) expression in peripheral blood mononuclear cells (PBMCs) and CD4+ T cells, and flow cytometry to detect DRD expression in Tfh cells. Finally, in vitro experiments and RNA sequencing (RNA-seq) were used to explore the possible pathway by which DRDs regulate Tfh cell differentiation. Results: The plasma dopamine concentration in patients with SLE was significantly increased, and abnormal mRNA expression of DRDs was observed in both PBMCs and CD4+ T cells. The results of flow cytometry showed that D1-like receptors were highly expressed in Tfh cells of patients with SLE and associated with disease activity. In vitro induction experiments showed that differentiation of naïve T cells into Tfh cells was accompanied by an increase in D1-like receptor expression. RNA-seq and RT-qPCR results indicate that D1-like receptors might promote Tfh cell differentiation through the Phosphatidylinositol3-kinase (PI3K)/protein kinase B (AKT)/Forkhead box protein O1 (FOXO1)/Kruppel-like factor 2 (Klf2) pathway. Conclusion: Tfh cells in patients with SLE highly express D1-like receptors, which correlate with disease activity. D1-like receptors may promote Tfh cell differentiation through the PI3K/AKT/FOXO1/Klf2 pathway.
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... The prevalence of SLE in mainland China is about 30-70/100,000, and the ratio of males to females is 1:10-12 (3). It is estimated that across all Hospital Authority (HA) hospitals in Hong Kong, there were 5243 patients with SLE in 2008, 91% of whom were women (4). The prevalence of SLE in Hong Kong was calculated to be 0.1%(4) Therapy-or disease-related comorbidities may occur in SLE patients. ...
A Framework on Developing an Integrative Medicine Clinical Practice Guideline (CPG) for Systemic Lupus erythematosus (SLE) with Comorbidities Management in Hong Kong
Preprint
Full-text available
  • Apr 2023
Background: Systemic Lupus erythematosus (SLE) is a heterogeneous autoimmune disease that affects multiple organs. In most patients with SLE, the comorbidity manifests within three years of initial diagnosis. In Hong Kong, SLE with comorbidities significantly affect patients' quality of life and contributes to mortality. Due to the complexity of comorbidities, clinicians require comprehensive suggestions across disciplines. An integrative medicine clinical practice guideline (CPG) must be developed for the management of SLE with comorbidities. Therefore, this framework is designed to address multimorbidity in SLE patients. Methods & Results: The framework is based on the steps of developing CPGs outlined in the WHO handbook for guideline development, Appraisal of Guidelines for Research and Evaluation II Instrument, Guideline International Network and Reporting Items for Practice Guidelines in Healthcare. The design of CPG for SLE includes nine steps as the followings: topic scoping, establishing a guideline development group, formulating key clinical questions, selecting and rating outcomes, conducting a systematic review of the evidence, assessing the quality of evidence, developing recommendations, drafting and publishing the guideline document, and updating are conducted. Conclusion: This CPG represents a significant advancement in establishing guidelines for multimorbidity in SLE. It will provide recommendations for the management of comorbidities in SLE. To update and adjust recommendations, further studies with large-scale clinical trials are necessary for the future.
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... Epidemiological studies suggest that the prevalence of SLE in Chinese populations was approximately 50-100/100 000 [23][24][25], whereas data from European and North America suggested that the prevalence of SLE in Caucasians was 10-35/100 000 [26,27]. Conversely, the prevalence of SSc was higher in Caucasians (8.8-30.5/100 ...
Clinical characteristics and prognosis of connective tissue disease-associated pulmonary arterial hypertension
Preprint
Full-text available
  • Nov 2022
Background Pulmonary arterial hypertension (PAH) is a series of clinicopathological syndromes associated with elevated pulmonary arterial pressure caused by various reasons, and is also the most serious complication of connective tissue disease (CTD). This study aims to evaluate the clinical features and prognostic factors of CTD associated PAH (CTD-PAH) patients in the Chinese Han population, and provide a reference basis for improving their prognosis. Methods A total of 392 individuals with CTD between January 2017 and August 2021 were included in this study. They were divided into two main groups: CTD-PAH and CTD-non-PAH. The independent sample t-test and Mann-Whitney U test were used to compare clinical and laboratory findings. Receiver operating characteristic (ROC) curves were converted into binary variables to determine the critical value. A Kaplan-Meier survival analysis was used to compare the survival rates of the two groups. And an analysis of death risk factors was conducted using Cox regression. Results In this study, SLE was the most common underlying disease among Chinese Han patients with CTD-PAH (51.56%). The level of anti-U1-RNP antibodies was significantly higher in the CTD-PAH group as compared to the CTD-non-PAH group (P = 0.001), whereas the levels of anti-SCL-70 antibodies were lower (P < 0.001). CTD patients with higher anti-U1-RNP antibodies levels were at high risk for PAH (P = 0.021), whereas higher levels of anti-U1-RNP have also been linked to decreased mortality in CTD-PAH (P < 0.001). In addition, the anti-SCL-70 antibody was an independent protective factor for CTD-PAH (P = 0.029). The poor prognosis of CTD-PAH was associated with high RDW (P = 0.010), NLR (P < 0.001), CAR (P = 0.001) and pulmonary infection (P = 0.008). Conclusion Patients with CTD who have higher levels of anti-U1-RNP antibodies are at high risk for PAH, whereas higher levels of anti-U1-RNP antibodies have also been linked to decreased mortality. An increase in the anti-SCL-70 antibody levels is an independent protective factor against CTD-PAH. High CAR, NLR, RDW and pulmonary infection are also significant determinants of total mortality in patients with CTD-PAH.
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Risk factors for mortality in systemic lupus erythematosus patients: Analysis of adult and pediatric cohorts in Taiwan
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  • Nov 2022
Background: Overall survival of systemic lupus erythematosus (SLE) patients significantly increased in recent decades, however, the relative risk of mortality is still high. Long-term survival outcome of pediatric SLE remains unclear. This study aims to explore the long-term survival rate and its predictors in patients with systemic lupus erythematosus (SLE). Methods: A retrospective, hospital-based cohort study was performed between 2004 and 2018 in a tertiary referral medical center in Taiwan. Data on comorbidities, medications, and causes of admission were collected for risk factor analysis using time-dependent multivariate Cox proportional hazards models. Results: A total of 2392 adults and 115 pediatric SLE patients were enrolled (female, n = 2157 and 95, respectively). The 10-year survival rates were 93.2%, 90.2%, 98.9%, and 100% in adult women, adult men, girls, and boys with SLE, respectively. The overall mortality rate was 2.09 case/100 patient-years (PY) for male SLE and 1.39 case/100 PY for female SLE patients. Male SLE patients did not have a statistically significantly higher mortality rate than female SLE patients in each age stratification. Infectious disease (n = 119), heart failure (n = 21), and cerebrovascular accident (n = 14) were the leading causes of death in adult SLE patients. Advanced age (hazard ratio [HR]: 1.04, 95% confidence interval [CI]: 1.03-1.05), treatment with mean dosage of systemic glucocorticoid equivalent to >10 mg/d of prednisolone (HR: 1.71, 95% CI: 1.14-2.57), comorbidities with malignancy (HR: 1.94, 95% CI: 1.22-3.09), chronic kidney disease (HR: 1.86, 95% CI: 1.25-2.77), hypertension (HR: 1.42, 95% CI: 1.01-1.98), and admission due to bacterial pneumonia (HR: 1.92, 95% CI: 1.12-3.31) and sepsis (HR: 2.78, 95% CI: 1.51-5.13) were independent risk factors for mortality in SLE patients. Conclusion: SLE patients with advanced age, malignancy, chronic kidney disease, hypertension, treated with a higher average dosage of glucocorticoids, and admission due to bacterial pneumonia and sepsis have an increased risk of mortality.
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Effect of Tacrolimus vs Intravenous Cyclophosphamide on Complete or Partial Response in Patients With Lupus Nephritis: A Randomized Clinical Trial
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Importance: Lupus nephritis (LN) is typically treated with intravenous cyclophosphamide (IVCY), which is associated with serious adverse effects. Tacrolimus may be an alternative for initial treatment of LN; however, large-scale, randomized clinical studies of tacrolimus are lacking. Objective: To assess efficacy and safety of tacrolimus vs IVCY as an initial therapy for LN in China. Design, setting, and participants: This randomized (1:1), open-label, parallel-controlled, phase 3, noninferiority clinical trial recruited patients aged 18 to 60 years with systemic lupus erythematosus and LN class III, IV, V, III+V, or IV+V primarily from outpatient settings at 35 centers in China. Inclusion criteria included body mass index of 18.5 or greater to less than 27, 24-hour urine protein of 1.5 g or greater, and serum creatinine of less than 260 μmol/L. Of 505 patients screened, 191 failed screening (163 ineligible, 25 withdrawn consent, and 3 other reasons). Overall, 314 were randomized. The first patient was enrolled March 10, 2015, and the study finished September 13, 2018. The follow-up period was 24 weeks. Data were analyzed from December 2019 to March 2020. Interventions: Oral tacrolimus (target trough level, 4-10 ng/mL) or IVCY for 24 weeks plus prednisone. Main outcomes and measures: Complete or partial response rate at week 24 (prespecified). Results: A total of 314 patients were randomized (158 [50.3%] to tacrolimus and 156 [49.7%] to IVCY). Overall, 299 patients (95.2%) were treated (tacrolimus group, 157 [52.5%]; IVCY group, 142 [47.5%]). Baseline demographic and clinical characteristics were generally similar between groups (mean [SD] age, 34.2 [9.5] years; 262 [87.6%] female). Tacrolimus was found to be noninferior to IVCY for LN response at week 24. There was a complete or partial response rate of 83.0% (117 of 141 patients) in the tacrolimus group and 75.0% (93 of 124 patients) in the IVCY group (difference, 7.1%; 2-sided 95% CI, -2.7% to 16.9%; lower limit of 95% CI greater than -15%). At week 24, least-square mean change in Systemic Lupus Erythematosus Disease Activity Index score was -8.6 with tacrolimus and -6.4 with IVCY (difference, -2.2; 95% CI, -3.1 to -1.3). Changes in other immune parameters and kidney function were generally similar between groups. Serious treatment-emergent adverse events (TEAEs) were reported in 29 patients in the tacrolimus group (18.5%) and 35 patients in the IVCY group (24.6%). Most common serious study drug-related TEAEs were infections (14 [8.9%] and 23 [16.2%], respectively). Seven patients in each group withdrew due to AEs. Conclusions and relevance: In this study, oral tacrolimus appeared noninferior to IVCY for initial therapy of active LN, with a more favorable safety profile than IVCY. Tacrolimus may be an alternative to IVCY as initial therapy for LN. Trial registration: ClinicalTrials.gov Identifier: NCT02457221.
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The aim of this study is to determine the risk and predictive factors for work disability in patients with SLE. A cross-sectional questionnaire study was performed to evaluate the employment status of a sample of consecutive Chinese patients with SLE. Demographic, socioeconomic data (age, gender, marital status, years of education and household income), employment status, self-reported fatigue score and disease characteristics (SLE duration, organ damage and disease activity) were collected. Work disability was defined by the failure to work due to SLE. The cumulative incidence of work disability since the time of SLE diagnosis was studied by a Kaplan Meier’s plot, and factors predictive of work disability were studied by Cox regression. A total of 147 patients with SLE were studied (mean age = 39.4 ± 11.3 years; 95% women). Among 105 patients who were working at the time of SLE diagnosis, 39 (37%) lost their ability to work as a result of SLE after a mean disease duration of 10.0 ± 6.1 years. Twenty-two (56%) patients lost their work ability within 2 years of diagnosis of SLE. The self-reported reasons for job loss were musculoskeletal pain (87%), skin disease (26%), renal problem (21%), fatigue (85%), memory deterioration (51%), anxiety or depressive symptoms (74%), too frequent sick leave (10%) and long-term hospitalisation (10%). The cumulative risk of work disability was 36% at 5 years after SLE diagnosis. In a Cox regression model, age (HR = 1.06 [1.02–1.11] per year; P = 0.008), self-reported fatigue score (HR = 1.06 [1.01–1.10] per point; P = 0.01) and mean disease activity score in the preceding two years (HR = 1.20 [1.02–1.42] per point; P = 0.03) were independently associated with working disability. In all, 37% of this group of patients with SLE lost their work ability after having the disease for 10 years. More than 50% of these patients developed work disability within the first 2 years of SLE diagnosis. Older age, fatigue and more active disease were independent predictors of work disability.
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A prospective study of survival and prognostic indicators of systemic lupus erythematous in a southern Chinese population
Article
Full-text available
  • May 2000
To study the survival rate and prognostic indicators of systemic lupus erythematosus (SLE) in a southern Chinese population. One hundred and eighty-six patients with SLE diagnosed between 1992 and 1999 were prospectively followed. Clinical features at presentation, subsequent evolving features, autoantibody profile, damage scores and mortality data were obtained. Prognostic factors for survival were studied by statistical analysis. One hundred and sixty-three female and 23 male SLE patients were studied. The female to male ratio was 7.1 to 1 and the mean age at presentation was 33.6 yr (range 12-75). The mean disease duration was 45.2 months. At diagnosis, arthritis, malar rash and alopecia were the commonest features. During follow-up, the prevalence of nephritis, arthritis, photosensitivity and haematological disease increased significantly. Thirty-one per cent of the patients had organ damage at the time of data analysis and renal disease was the commonest cause. Logistic regression revealed that central nervous system disease, discoid lesions and treatment with high-dose steroid were independent predictors for damage. Nine patients died during the study period (three of disease-related complications and six of infections). The 3-, 5-, and 7-yr survival rates of our cohort were 97, 93 and 93%, respectively. Cox regression analysis revealed that thrombocytopenia and high-dose steroid treatment were independent risk factors for mortality. The survival of SLE in our southern Chinese patients is similar to that of the Caucasian series reported in the 1990s. Although nephritis contributes to organ damage, it is not a major determinant for survival. Infection remains the commonest cause of death. High-dose steroid treatment and thrombocytopenia are independent risk factors for mortality. Judicious use of immunosuppressive agents is necessary to improve the short-term survival of SLE.
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Childhood-Onset Disease as a Predictor of Mortality in an Adult Cohort of Patients With Systemic Lupus Erythematosus
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To examine childhood-onset disease as a predictor of mortality in a cohort of adult patients with systemic lupus erythematosus (SLE). Data were derived from the University of California Lupus Outcomes Study, a longitudinal cohort of 957 adult subjects with SLE that includes 98 subjects with childhood-onset SLE. Baseline and followup data were obtained via telephone interviews conducted in 2002-2007. The number of deaths during 5 years of followup was determined and standardized mortality ratios (SMRs) for the cohort, and across age groups, were calculated. Kaplan-Meier life table analysis was used to compare mortality rates between childhood- (defined as SLE diagnosis at <18 years of age) and adult-onset SLE. Multivariate Cox proportional hazard models were used to determine predictors of mortality. During the median followup period of 48 months, 72 deaths (7.5% of subjects) occurred, including 9 deaths (12.5%) in subjects with childhood-onset SLE. The overall SMR was 2.5 (95% confidence interval [95% CI] 2.0-3.2). In Kaplan-Meier survival analysis, after adjusting for age, childhood-onset subjects were at increased risk for mortality throughout the followup period (P< 0.0001). In a multivariate model adjusting for age, disease duration, and other covariates, childhood-onset SLE was independently associated with an increased mortality risk (hazard ratio [HR] 3.1, 95% CI 1.3-7.3), as was low socioeconomic status measured by education (HR 1.9, 95% CI 1.1-3.2), and end stage renal disease (HR 2.1, 95% CI 1.1-4.0). Childhood-onset SLE was a strong predictor of mortality in this cohort. Interventions are needed to prevent early mortality in this population.
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Distinct patterns of systemic lupus erythematosus identified by cluster analysis
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The objective of this study was to evaluate the patterns of clinical manifestations and their mortality in a large cohort of Chinese patients with systemic lupus erythematosus. The cumulative clinical manifestations of a large group of Chinese systemic lupus erythematosus patients who fulfilled at least four American College of Rheumatology criteria for systemic lupus erythematosus were studied. Patients were divided into distinct groups by using the K-mean cluster analysis. Clinical features, prevalence of proliferative lupus nephritis (World Health Organization class III, IV), autoantibody profile, and treatment data were compared and the standardized mortality ratios were calculated for each cluster of patients. There were 1082 patients included in the study (mean age at systemic lupus erythematosus diagnosis 30.5 years; mean systemic lupus erythematosus duration 10.3 years). Three distinct groups of patients were identified. Cluster 1 (n = 347) was characterized predominantly by mucocutaneous manifestations (malar rash, discoid rash, photosensitivity, oral ulcer) and arthritis but having the lowest prevalence of serositis, hematologic manifestations (hemolytic anemia, leukopenia, and thrombocytopenia), and proliferative lupus nephritis. Patients in cluster 2 (n = 409) had mainly renal and hematological manifestations but having the lowest prevalence of mucocutaneous manifestations. Pulmonary and gastrointestinal manifestations were significantly more frequent in cluster 2 than the other clusters. Cluster 3 patients (n = 326) had the most heterogeneous features. Besides having a high prevalence of mucocutaneous manifestations, serositis and hematologic manifestations, renal involvement, and proliferative lupus nephritis was also most prevalent among the three clusters. Patients in cluster 2 had a much higher standardized mortality ratio [standardized mortality ratio 7.23 (6.7-7.7), p < 0.001] than those in cluster 3 [standardized mortality ratio 1.27 (1.1-1.5), p = 0.005] and cluster 1 [standardized mortality ratio 0.95 (0.5-1.7), p = 0.86]. In conclusion, patients with systemic lupus erythematosus could be clustered into prognostically distinct patterns of clinical manifestations.
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The 1000 Canadian Faces of Lupus: Determinants of Disease Outcome in a Large Multiethnic Cohort
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To describe disease expression and damage accrual in systemic lupus erythematosus (SLE), and determine the influence of ethnicity and socioeconomic factors on damage accrual in a large multiethnic Canadian cohort. Adults with SLE were enrolled in a multicenter cohort. Data on sociodemographic factors, diagnostic criteria, disease activity, autoantibodies, treatment, and damage were collected using standardized tools, and results were compared across ethnic groups. We analyzed baseline data, testing for differences in sociodemographic and clinical factors, between the different ethnic groups, in univariate analyses; significant variables from univariate analyses were included in multivariate regression models examining for differences between ethnic groups, related to damage scores. We studied 1416 patients, including 826 Caucasians, 249 Asians, 122 Afro-Caribbeans, and 73 Aboriginals. Although the overall number of American College of Rheumatology criteria in different ethnic groups was similar, there were differences in individual manifestations and autoantibody profiles. Asian and Afro-Caribbean patients had more frequent renal involvement and more exposure to immunosuppressives. Aboriginal patients had high frequencies of antiphospholipid antibodies and high rates of comorbidity, but disease manifestations similar to Caucasians. Asian patients had the youngest age at onset and the lowest damage scores. Aboriginals had the least education and lowest incomes. The final regression model (R2=0.27) for higher damage score included older age, longer disease duration, low income, prednisone treatment, higher disease activity, and cyclophosphamide treatment. There are differences in lupus phenotypes between ethnic populations. Although ethnicity was not found to be a significant independent predictor of damage accrual, low income was.
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Effect of disease activity and damage on quality of life in patients with systemic lupus erythematosus: A 2-year prospective study
Article
  • Dec 2008
  • SCAND J RHEUMATOL
To examine the effect of disease activity and damage on health-related quality of life (HRQoL) in patients with systemic lupus erythematosus (SLE). Consecutive SLE patients and matched controls were recruited for a study of HRQoL using the Medical Outcomes Study Short Form-36 (SF-36). SLE activity and damage was assessed by the Safety of Oestrogens in Lupus Erythematosus National Assessment SLE Disease Activity Index (SELENA-SLEDAI) and the American College of Rheumatology/Systemic Lupus International Collaborating Clinics (ACR/SLICC) Damage Index (SDI), respectively. Patients were prospectively followed for repeat HRQoL assessment at 2 years. The effects of cumulative disease activity and new damage on changes in SF-36 scores were evaluated. One hundred and fifty-five patients were studied (94% women; age 37.8+/-11.3 years; SLE duration 7.2+/-5.4 years). Fifty (32%) patients had active disease and 75 (48%) had organ damage at baseline. Compared with age- and gender-matched controls, SLE patients had lower SF-36 scores, and the difference remained significant after adjustment for income and education level. SF-36 scores in SLE patients correlated inversely with SDI but not with SELENA-SLEDAI scores. After 2 years, there was a significant drop in the mental component score of the SF-36. Regression analysis revealed that new damage was the only determinant for a reduction in SF-36 scores. Patients with higher cumulative disease activity had a greater drop in bodily pain and general health subscores. Impaired HRQoL is more common in SLE patients than controls, regardless of age, sex, education and poverty. Pre-existing organ damage is associated with poorer HRQoL and new damage predicts a further decline in HRQoL. Persistent disease activity is associated with deterioration in certain domains of the SF-36.
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Changing Patterns in Mortality and Disease Outcomes for Patients with Systemic Lupus Erythematosus
Article
  • Oct 2008
Survival of patients with systemic lupus erythematosus (SLE) has improved significantly, but new morbidities have emerged, leading to altered patterns of outcome in this disease. We examined changes in mortality and other outcomes over time in a large SLE cohort. A group of 1241 patients from the University of Toronto Lupus Clinic followed prospectively were divided into 4 entry cohorts - 1: 1970-1978, 2: 1979-1987, 3: 1988-1996, 4: 1997-2005. These cohorts were followed through four 9-year calendar periods defined over the same intervals. Both cohort and calendar effects were assessed for the following outcomes: mortality (standardized mortality ratio; SMR), disease activity over time (adjusted mean SLEDAI; AMS), cumulative damage (Systemic Lupus International Collaborating Clinics Damage Index; SDI), coronary artery disease (CAD), and osteonecrosis (ON). Cox regression models were used to further investigate mortality and the influence on it of the disease-related factors. Over the 36-year period of the study, 211 deaths occurred. The overall SMR in the first and last decades were 12.60 (95% CI: 9.13, 17.39) and 3.46 (95% CI: 2.71, 4.40) respectively. When SMR were stratified by the entry cohort and calendar period, there is evidence of a calendar-period effect but no cohort effect. The AMS decreased over the decades, while SDI, CAD, and ON increased. There were significant detrimental effects for male sex, CAD, AMS, SDI, and use of immunosuppressive drugs and significant protective effects for use of antimalarials and the effect of calendar period on mortality. Our study demonstrates improved survival in patients with SLE over a 36-year period. Disease-related variables included in the model are important factors for mortality in this SLE cohort, but could not completely explain the trend of improved survival over calendar period observed.
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Incidence and mortality of systemic lupus erythematosus in a southern Chinese population, 2000-2006
Article
  • Aug 2008
To study the annual incidence and standardized mortality ratio (SMR) of a longitudinal cohort of Chinese patients with systemic lupus erythematosus (SLE). Annual numbers of new cases and deaths in a longitudinal cohort of patients with SLE between 2000 and 2006 retrieved from a database were compared with regional population and death rates expected from the annual death statistics maintained by our hospital and population census data. Our cohort of SLE had grown from 272 to 442 patients from 2000 to 2006. The annual incidence of SLE showed mild fluctuation (mean incidence 3.1/100,000 population; 5.4/100,000 in women). The annual death rate and SMR in year 2000 were 25.7/1000 and 7.88 (range 3.7-16.7; p<0.001), respectively, compared to the general population. A trend of reduction in annual death rates and SMR was observed, the annual death rate and SMR in year 2006 being 6.8/1000 and 2.17 (range 0.7-6.7; p=0.34). The SMR was higher in men than women and had a less obvious trend of improvement. A negative correlation of SMR with age was observed. The SMR of SLE patients aged above 60 years was not significantly higher than expected from population statistics. There was also a trend of fewer deaths due to infection over time. In this single-center study, the incidence of SLE remained static. The SMR of SLE was significantly increased in younger patients, indicating a greater effect of the disease on younger individuals. There was a trend of improvement in SMR for SLE in recent years, probably as a result of fewer infectious complications.
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Mortality in systemic lupus erythematosus: The bimodal pattern revisited
Article
  • May 1985
  • QJM
A review of 51 patients who died while enrolled in a long-term prospective study of systemic lupus erythematosus (SLE) revealed that active SLE may persist or reappear late in the course of the disease. Vascular events, especially atherosclerotic coronary artery disease, occurred frequently. Moderate to severe atherosclerosis was seen in patients who had died of any cause after a prolonged duration of the disease and often contributed significantly to death. Diffuse proliferative glomerulonephritis, CNS lupus and major infections were indications of poor prognosis particularly early in its course.
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Epidemiology of systemic lupus erytematosus
Article
  • Jan 2003
  • BEST PRACT RES CL RH
Several recent studies have suggested that the incidence of systemic lupus erythematosus (SLE) is increasing. The female to male ratio varies from 4.3 to 13.6. SLE is more common in African-Americans, African-Caribbeans, and Asians, than in Caucasians. The age at diagnosis is younger in African-Americans than in Caucasians. Improvement in survival levelled off in the 1980s; 20-year survival is only 53-61%.
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