Differences in the Clinical Manifestations and Mortality of Systemic Lupus Erythematosus Onset in Children and Adults: A Systematic Review and Meta-Analysis

Author(s):  
Xiaolan Huang ◽  
Nan Jia ◽  
Fei Xiao ◽  
Chunrong Sun ◽  
Jia Zhu ◽  
...  

<b><i>Introduction:</i></b> The aim of this study was to assess the differences between childhood-onset and adult-onset systemic lupus erythematosus (cSLE and aSLE) for clinical manifestations and mortality using a meta-analytic approach. <b><i>Methods:</i></b> The PubMed, EMBASE, and the Cochrane library were searched for eligible studies published between January 1982 and March 2021. The odds ratio (OR) with 95% confidence interval was used to calculate the pooled effect estimates using the random-effects model. <b><i>Results:</i></b> Thirty-four studies involving 21,946 SLE patients were included. cSLE was associated with an increased risk of malar rash (OR: 1.64; <i>p</i> &#x3c; 0.001), ulcers/mucocutaneous involvement (OR: 1.22; <i>p</i> = 0.039), general neurological involvement (OR: 1.52; <i>p</i> &#x3c; 0.001), seizures (OR: 1.92; <i>p</i> &#x3c; 0.001), general renal involvement (OR: 2.08; <i>p</i> &#x3c; 0.001), proteinuria (OR: 1.35; <i>p</i> = 0.015), urinary cellular casts (OR: 1.67; <i>p</i> = 0.047), fever (OR: 2.31; <i>p</i> &#x3c; 0.001), anemia (OR: 1.91; <i>p</i> &#x3c; 0.001), thrombocytopenia (OR: 1.41; <i>p</i> &#x3c; 0.001), leucopenia (OR: 1.57; <i>p</i> = 0.017), lymphadenopathy (OR: 2.40; <i>p</i> &#x3c; 0.001), and cutaneous vasculitis (OR: 1.72; <i>p</i> = 0.001) as compared with aSLE. Moreover, cSLE versus aSLE was associated with a reduced risk of articular manifestations (OR: 0.63; <i>p</i> = 0.001), pulmonary involvement (OR: 0.54; <i>p</i> = 0.001), and pleuritis (OR: 0.61; <i>p</i> &#x3c; 0.001). There were no significant differences between cSLE and aSLE for mortality risk (OR: 1.20; <i>p</i> = 0.203). <b><i>Conclusion:</i></b> We found that certain clinical manifestations of SLE are different in cSLE and aSLE. Moreover, the mortality risk of cSLE and aSLE was not significantly different.

2021 ◽  
Vol 61 (1) ◽  
Author(s):  
Yu Fu ◽  
Qing Lin ◽  
Zhi-rong Zhang

Abstract Objective To more precisely estimate the association between the tumor necrosis factor ligand superfamily member 4 (TNFSF4) gene polymorphisms and systemic lupus erythematosus (SLE) susceptibility, we performed a meta-analysis on the association of the following single nucleotide polymorphisms (SNPs) of TNFSF4 with SLE: rs1234315, rs844648, rs2205960, rs704840, rs844644, rs10489265. Methods A literature-based search was conducted using PubMed, MEDLINE, Embase, Web of Science databases, and Cochrane Library databases to identify all relevant studies. And the association of TNFSF4 gene polymorphisms and SLE susceptibility was evaluated by pooled odds ratio (OR) with 95% confidence interval (CI). Results The meta-analysis produced overall OR of 1.42 (95% CI 1.36–1.49, P < 0.00001), 1.41 (95% CI 1.36–1.46, P < 0.00001) and 1.34 (95% CI 1.26–1.42, P < 0.00001) for the rs2205960, rs1234315 and rs704840 polymorphisms respectively, confirming these three SNPs confer a significant risk for the development of SLE. On the other hand, the meta-analysis produced overall OR of 0.92 (95% CI 0.70–1.21, P = 0.54) for the rs844644 polymorphism, suggesting no significant association. And no association was also found between either rs844648 1.11 (OR 1.11, 95% CI 0.86–1.43, P = 0.41) or rs10489265 (OR 1.17, 95% CI 0.94–1.47, P = 0.17) polymorphism with SLE susceptibility, respectively. Conclusions Our meta-analysis demonstrated that the TNFSF4 rs2205960, rs1234315 and rs844840 SNPs was significantly associated with an increased risk of SLE.


Lupus ◽  
2020 ◽  
pp. 096120332097408
Author(s):  
Zhao Jin ◽  
Cong Yang ◽  
Chu Xiao ◽  
Zizhen Wang ◽  
Suxin Zhang ◽  
...  

Objective To systematically review and summarize the available literature regarding the association between systemic lupus erythematosus (SLE) and sexual dysfunction (SD) in both sexes. Methods We retrieved relevant studies from the following databases: PubMed, Embase, Cochrane Library, and Web of Science. Two reviewers independently reviewed the studies in our sample, assessed their validity, and extracted relevant data. Sensitivity and subgroup analyses were performed to distinguish sources of heterogeneity. Results Our search resulted in a sample of eight eligible studies, which involved 758 patients in the SLE group and 1724 individuals in the control group. The pooled RR for the increased risk for SD compared to those in the control group was 1.80 (95%CI 1.12-2.87). Subgroup analysis by sex revealed that males (pooled RR = 2.98, 95%CI 2.41-3.68) had a higher risk of SD compared to females (pooled RR = 1.56, 95%CI 0.99-2.48). Females with SLE had significantly lower values in FSFI compared to the healthy individuals (WMD=-0.224, 95%CI -0.441 to -0.078). Age of participants and the quality of studies might influence the results. Conclusions Our meta-analysis suggests that SLE is significantly associated with an increased risk of sexual dysfunction. It is of great urgency to implement for active interventions that aimed to treat or prevent SD among SLE patients.


Rheumatology ◽  
2021 ◽  
Author(s):  
Lanlan Ji ◽  
Wenhui Xie ◽  
Zhuoli Zhang

Abstract Objective To assess the risk of flare and damage accrual after low dose glucocorticoids (GC) discontinuation in systemic lupus erythematosus (SLE). Methods We performed a comprehensive literature search of PubMed, EMBASE, Cochrane library and Scopus databases from inception to July 2020 for studies concerning relapses/damage accrual in SLE patients. Pooled incidence rates of flare and time to flare with their 95% confidence intervals (CI) after GC withdrawal were calculated. Summary risk ratio (RR) and 95% CI of flare/organ damage accrual risk were computed using a random or fixed effects model. Results 738 SLE patients with GC discontinuation in 17 publications were eligible for the final analysis. In the primary meta-analysis, the pooled incidence of flare was 24% (95% CI 21-27%) and 13% (95% CI 8-18%) for global and major flare respectively. Pooled time to flare was 21.08 (95% CI 9.32-32.85) months. In the secondary meta-analysis, GC discontinuation showed an increased risk of flare comparing with GC continuation [RR (95% CI) =1.38 (1.01-1.89)], but the risk of major flares was not increased (RR = 1.77, 95% CI 0.40-7.83). Moreover, GC withdrawal was associated with a borderline reduction of risk in SDI increase in comparison with GC continuation (RR = 0.64, 95% CI 0.38 - 1.09). Conclusion GC discontinuation leads to a slightly increased risk of flare, however no increase in major flare and a borderline reduction of risk in further damage in SLE patients. Baseline screening for candidate patients and long-term follow up after GC withdrawal are needed to reliably evaluate the organ damage increase.


BMJ Open ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. e031850 ◽  
Author(s):  
Irene B Murimi-Worstell ◽  
Dora H Lin ◽  
Henk Nab ◽  
Hong J Kan ◽  
Oluwadamilola Onasanya ◽  
...  

ObjectiveAt least half of patients with systemic lupus erythematosus (SLE) develop organ damage as a consequence of autoimmune disease or long-term therapeutic steroid use. This study synthesised evidence on the association between organ damage and mortality in patients with SLE.DesignSystematic review and meta-analysis.MethodsElectronic searches were performed in PubMed, Embase, Cochrane Library and Latin American and Caribbean Health Sciences Literature for observational (cohort, case-control and cross-sectional) studies published between January 2000 and February 2017. Included studies reported HRs or ORs on the association between organ damage (measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score) and mortality. Study quality was assessed using the modified Newcastle-Ottawa assessment. Pooled HRs were obtained using the DerSimonian and Laird random-effects model. Heterogeneity was assessed using the Cochrane Q (Q) and I2 statistics.ResultsThe search yielded 10 420 articles, from which 21 longitudinal studies were selected. Most studies (85%) were of high quality. For 10 studies evaluating organ damage (SDI) as a continuous variable and reporting HR as a measure of association, a 1-unit increase in SDI was associated with increased mortality; pooled HR was 1.34 (95% CI: 1.24 to 1.44, p<0.001; Q p=0.027, I2=52.1%). Exclusion of one potential outlying study reduced heterogeneity with minimal impact on pooled HR (1.33 (95% CI: 1.25 to 1.42), p<0.001, Q p=0.087, I2=42.0%). The 11 remaining studies, although they could not be aggregated because of their varying patient populations and analyses, consistently demonstrated that greater SDI was associated with increased mortality.ConclusionsOrgan damage in SLE is consistently associated with increased mortality across studies from various countries. Modifying the disease course with effective therapies and steroid-sparing regimens may reduce organ damage, improve outcomes and decrease mortality for patients with SLE.


Lupus ◽  
2020 ◽  
Vol 29 (10) ◽  
pp. 1216-1226
Author(s):  
Beatriz Frade-Sosa ◽  
Javier Narváez ◽  
Tarek Carlos Salman-Monte ◽  
Raul Castellanos-Moreira ◽  
Vera Ortiz-Santamaria ◽  
...  

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.


2011 ◽  
Vol 38 (9) ◽  
pp. 1914-1919 ◽  
Author(s):  
LI-HSIN LIN ◽  
PIN LING ◽  
MING-FEI LIU

Objective.Type I interferons (IFN), especially IFN-α, have been proposed to underlie the pathogenesis of systemic lupus erythematosus (SLE). Members of the IFN regulatory factor (IRF) family, which regulate IFN expression, have been implicated as risk factors for SLE. Our aims were to investigate the expression of IRF7 and its correlation with disease activity and to explore the association in Taiwanese patients between 2 genetic single-nucleotide polymorphisms (SNP) of IRF7 and SLE.Methods.IRF7 messenger RNA (mRNA) levels were measured in peripheral blood mononuclear cells by real-time reverse transcription polymerase chain reaction in 51 adult patients with SLE and 65 age-matched and sex-matched controls. Their serum IFN-α levels were determined by ELISA and the clinical manifestations were recorded at the same time. Two IRF7 SNP, rs1061501 and rs1061502, were examined by genotyping across 92 patients with SLE and 92 age and sex-matched healthy control subjects.Results.Compared with controls, the expression of IRF7 mRNA was significantly increased in patients with SLE and was positively correlated with both the serum level of IFN-α and lupus disease activity. The distribution of SNP rs1061501 by genotype (CC, CT, and TT) and by allele (C, T) was significantly different between the SLE and the control group (p = 0.028 for genotype and p = 0.009 for allele). There were no significant differences for SNP rs1061502.Conclusion.The results suggest that dysregulation of IRF7 might mediate an excessive production of IFN-α, which then exerts a crucial effect on the pathogenesis of human SLE. The IRF7 SNP rs1061501 TT genotype and T allele are enriched in Taiwanese patients with SLE and thus would seem to be associated with an increased risk of developing SLE.


2018 ◽  
Vol 12 (2) ◽  
pp. 143-151 ◽  
Author(s):  
Zhuoxian Zhao ◽  
Natalia P. Rocha ◽  
Haitham Salem ◽  
Breno S. Diniz ◽  
Antonio L. Teixeira

Abstract A growing body of evidence indicates that systemic lupus erythematosus (SLE) is associated with increased risk of cognitive impairment and dementia. However, to date, no studies have been conducted to quantitatively summarize and evaluate the consistency of data. Objective: To quantitatively evaluate the relationship of SLE and antiphospholipid antibodies (aPL) with cognitive dysfunction and dementia. Methods: All relevant literature was retrieved from Pubmed, Scopus, and PsycINFO databases. The meta-analysis was performed using effect estimates and 95% confidence intervals (CIs) to calculate pooled risk estimates. The heterogeneity among studies was also examined. Results: The meta-analysis included 11 original studies involving a total of 81,668 patients with dementia and 407 patients with cognitive dysfunction. There were significant associations on fixed-effect models between SLE and dementia (3 studies; RR=1.50; 95% CI=1.37-1.64), SLE and cognitive dysfunction (4 studies; OR=2.97; 95% CI=1.72-5.15), and aPL and cognitive dysfunction (5 studies, OR=1.97; 95% CI=1.55-2.52). We also combined cognitive dysfunction and dementia outcomes as they both represented cognitive impairment. There were significant associations between aPL and cognitive impairment (6 studies; OR=2.03; 95% CI=1.62-2.55), and SLE and cognitive impairment (7 studies; OR=1.83; 95% CI=1.42-2.35). Moderate heterogeneity (I2=45.7%) was found in the association between SLE and cognitive impairment, low heterogeneity (I2=21.8%) in the association between SLE and dementia, and near zero heterogeneity for the other three main analyses. Conclusion: Both SLE and aPL are associated with cognitive impairment.


Lupus ◽  
2010 ◽  
Vol 19 (12) ◽  
pp. 1365-1373 ◽  
Author(s):  
E. Osio-Salido ◽  
H. Manapat-Reyes

In this paper we aim to review the prevalence, incidence and disease presentation of lupus patients in Asian populations. The database of the National Library of Medicine (MEDLINE) through PUBMED up to September 2009 was searched for relevant articles using the search terms ‘systemic lupus erythematosus’, ‘epidemiology’, and ‘Asia’. Articles on lupus prevalence, incidence, and clinical manifestations were retrieved and their bibliographies further screened for relevant articles. Proceedings of national, regional, and international rheumatology and SLE conferences were likewise searched for relevant abstracts. Retrieval rate of relevant articles was approximately 80%. Prevalence and incidence figures are expressed per 100,000 populations. Among the articles reviewed, epidemiologic information was usually obtained through population surveys or hospital cohorts. Prevalence data are available from 24 countries, and generally fall within 30—50/100,000 population. However, one survey showed a higher prevalence of 70 (Shanghai), while three others showed a lower prevalence of 3.2—19.3 (India, Japan, Saudi Arabia). Only three countries have recorded incidence rates and these varied from 0.9 to 3.1 per annum. Thirty articles from 21 countries describe lupus disease presentation. Common manifestations include mucocutaneous lesions (seen in 52—98% of patients) and arthritis/musculoskeletal complaints (36—95%). Antinuclear antibodies were generally positive in 89—100% of patients, except for two studies. Renal involvement ranged from 18% to 100% with most articles reporting this in >50% of their patients. Discoid lesions, serositis, and neurologic involvement were the least frequently seen symptoms. There is varying epidemiologic information regarding systemic lupus erythematosus among countries in Asia. Prevalence rates usually fall within 30—50/100,000 population. Incidence rates, as reported from three countries, varied from 0.9/100,000 to 3.1% per annum. It is difficult to make generalizations about how the epidemiologic character of the disease varies from country to country. However, similarities in disease manifestations can be observed. .


Lupus ◽  
2017 ◽  
Vol 27 (3) ◽  
pp. 428-435 ◽  
Author(s):  
Y H Lee ◽  
G G Song

Objective We aimed to evaluate the relationship between circulating leptin levels and systemic lupus erythematosus (SLE). Methods MEDLINE, EMBASE, and Cochrane library databases were searched. Meta-analyses were performed comparing serum/plasma leptin levels in patients with SLE and healthy controls, and on patients with SLE in subgroups based on ethnicity, sample size, data type, and matched variables (age, sex, and/or body mass index (BMI)). Results Eighteen studies including 1333 patients with SLE and 1048 controls were ultimately selected, which showed that leptin levels were significantly higher in the SLE group than in the control group (SMD = 0.611, 95% CI = 0.275–0.947, p < 0.001). When we excluded two outlier studies because of high heterogeneity, leptin levels were also significantly higher in the SLE group than in the control group (SMD = 0.619, 95% CI = 0.431–0.807, p < 0.001). Stratification by ethnicity showed significantly elevated leptin levels in the SLE group in European, Asian, Arab, Latin American, and mixed populations. Subgroup analysis by sample size showed significantly higher leptin levels in the SLE group by small ( n ≤ 100) and large sample numbers ( n > 100) (SMD = 0.780, 95% CI = 0.445–1.115, p < 0.001; SMD = 0.495, 95% CI = 0.275–0.715, p < 0.001). Stratification by data type revealed significantly higher leptin levels in the original data and imputed data groups. Subgroup analysis adjustment revealed significantly higher leptin levels in the SLE group, regardless of adjustment for variables. Conclusions Our meta-analysis demonstrated that leptin levels were significantly higher in patients with SLE, regardless of ethnicity, sample size, data type, and matched variables.


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