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1.
MMWR Morb Mortal Wkly Rep ; 68(18): 419-422, 2019 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-31071073

RESUMO

Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease with often nonspecific symptoms that can lead to a delay in diagnosis. The disease disproportionately affects women and minorities. Blacks with SLE also have more severe disease and develop it at an earlier age (1). Despite an increase in the 5-year survival rate from 50% in 1955 to approximately 90% in the 2000s, attributed largely to advances in management of SLE (2), premature mortality among SLE patients persists, often as a result of disease severity, infections, and cardiovascular disease. Because existing SLE mortality estimates based on death certificate data are known to underestimate SLE deaths (3), SLE mortality was analyzed using 2002-2004 data from the population-based Georgia Lupus Registry (1). Incident and prevalent SLE cases matched to the National Death Index through 2016 identified 97 and 401 deaths, respectively. Standardized mortality ratios adjusted for age group, sex, and race were two to three times higher among persons with SLE relative to expected deaths in the general population. Blacks had significantly higher cumulative mortality than did whites, and blacks with both incident and prevalent cases were significantly younger at death (mean age 51.8 and 52.3 years, respectively) than were whites (mean age 64.4 and 65.0 years, respectively). Whites had lower mortality after diagnosis than did blacks; among incident cases, mortality among whites did not occur until 5 years after SLE diagnosis, whereas blacks had significantly and persistently higher mortality from the time of diagnosis. There were no significant differences by sex. Current CDC-supported efforts encourage early detection, diagnosis, and treatment, and enhanced self-management skills to mitigate racial disparities and improve outcomes overall among persons with SLE.


Assuntos
Afro-Americanos/estatística & dados numéricos , Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Disparidades nos Níveis de Saúde , Lúpus Eritematoso Sistêmico/etnologia , Mortalidade/etnologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Georgia/epidemiologia , Humanos , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Sistema de Registros
2.
Inflamm Res ; 68(8): 705-713, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31134304

RESUMO

OBJECTIVES: The BLK and BANK1 genes have been consistently associated with systemic lupus erythematosus (SLE), primarily in European or Asian-derived populations. However, this finding has not been replicated in Latin-American patients. METHODS: Our study included 881 women from Mexico: 487 healthy controls and 394 SLE patients. The BLK rs13277113A/G-rs2736340T/C as well as BANK1 rs10516487G/A (R61H)-rs3733197G/A (A383T) single nucleotide polymorphisms (SNPs) were evaluated using a TaqMan® SNP genotyping assay. RESULTS: Our data showed that the BLK rs2736340T/C and rs13277113A/G polymorphisms are associated with susceptibility to SLE (C vs T, OR 1.60, p = 2×10-5; G vs A, OR 1.53, p = 9 × 10-5, respectively). We also identified an association between the functional BANK1 R61H polymorphism and SLE (A vs G, OR 1.56, p = 0.002). In addition, we observed a genetic interaction between BLK (rs2736340T/C, rs13277113A/G) and BANK1 (R61H and A383T) associated with susceptibility to SLE. CONCLUSION: This is the first study documenting an association between BLK and BANK1 and SLE in a Latin-American population. Our data confirm previous reports: BLK and BANK1 are factors associated with SLE. Thus, both genes are universal loci for this autoimmune disease.


Assuntos
Proteínas Adaptadoras de Transdução de Sinal/genética , Lúpus Eritematoso Sistêmico/genética , Proteínas de Membrana/genética , Quinases da Família src/genética , Adulto , Feminino , Predisposição Genética para Doença , Humanos , Lúpus Eritematoso Sistêmico/etnologia , México , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo Único
3.
Lupus ; 28(7): 862-867, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31122136

RESUMO

OBJECTIVES: Hydroxychloroquine (HCQ) is a key therapy in systemic lupus erythematosus (SLE). Medication non-adherence is reported in up to 80% of lupus patients and results in increased morbidity, mortality, and health care utilization. HCQ levels are a sensitive and reliable method to assess medication adherence. Our study evaluated the role of HCQ level measurement in routine clinical care and its association with disease activity in a predominantly Hispanic population. METHODS: SLE patients from the Columbia University Lupus cohort treated with HCQ for ≥ 6 months and reporting medication adherence were included. HCQ levels were measured by whole blood high performance liquid chromatography. Non-adherence was defined as an HCQ level <500 ng/ml. The association between HCQ levels and disease activity measured by Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was evaluated. RESULTS: One hundred and eight patients were enrolled; the median age was 38 years, 91% were female, and 63% were Hispanic. The median SLEDAI-2K was 4.3 (0-20). Forty-one percent of patients had an HCQ level <500 ng/ml consistent with non-adherence, of which 19% had undetectable levels. A higher SLEDAI-2K score was associated with low HCQ levels (p = 0.003). This association remained significant after adjusting for depression (p = 0.0007). CONCLUSION: HCQ levels < 500 ng/ml were associated with higher disease activity and accounted for 32% of the SLEDAI-2K variability. HCQ blood measurement is a simple and reliable method to evaluate medication adherence in SLE. Reasons for non-adherence (levels < 500 ng/ml) should be further explored and addressed.


Assuntos
Antirreumáticos/sangue , Hidroxicloroquina/sangue , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adesão à Medicação , Adulto , Idoso , Antirreumáticos/uso terapêutico , Cromatografia Líquida de Alta Pressão , Estudos de Coortes , Esquema de Medicação , Monitoramento de Medicamentos , Feminino , Hispano-Americanos , Humanos , Hidroxicloroquina/uso terapêutico , Modelos Lineares , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Resultado do Tratamento , Estados Unidos , Adulto Jovem
4.
J Immunol Res ; 2019: 1547578, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30984790

RESUMO

Aims: This study is aimed at exploring the relation between IL-33 single-nucleotide polymorphisms (SNPs) and the risk of systemic lupus erythematosus (SLE). Methods: SNPStats (online software) was used to test the Hardy-Weinberg equilibrium in controls. Generalized multifactor dimensionality reduction (GMDR) was adopted to screen the preferable interaction between IL-33 SNPs and current smoking. Results: Logistic regression analysis based on the fundamental data of age, gender, BMI, current smoking, and alcohol drinking showed that both rs1929992-G and rs1891385-C alleles were correlated with an increasing risk of SLE, the ORs (95% CI) of which were 1.62 (1.21-2.05) and 1.64 (1.22-2.10), respectively. One two-locus model (rs1929992×current smoking) had a testing accuracy of 60.11% (P = 0.0010). Through an overall multidimensional model, optimum cross-validation consistency was obtained. The analysis indicated that current smoking status influenced the SLE risk depending on the genotypes at rs1929992. Pairwise LD analysis indicated that haplotype rs1929992G-rs7044343T was statistically related to the elevating risk of SLE (P < 0.05). Those subjects with the G-T haplotype had a higher SLE risk than those with other haplotypes, after correction with factors, including gender, alcohol drinking, age, BMI, and current smoking. Conclusions: The rs1929992-G and rs1891385-C allele, interaction between the rs1929992 gene and current smoking, and haplotype rs1929992G-rs7044343T were all risk factors of SLE.


Assuntos
Predisposição Genética para Doença , Interleucina-33/genética , Lúpus Eritematoso Sistêmico/genética , Polimorfismo de Nucleotídeo Único , Fumar/efeitos adversos , Adulto , Consumo de Bebidas Alcoólicas , Alelos , Grupo com Ancestrais do Continente Asiático , Feminino , Frequência do Gene , Estudos de Associação Genética , Genótipo , Haplótipos , Humanos , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Pessoa de Meia-Idade , Análise de Regressão , Fatores de Risco
5.
Lupus ; 28(4): 445-454, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30744525

RESUMO

BACKGROUND: The contribution of regulatory T-cells (Tregs) to systemic lupus erythematosus (SLE) pathogenesis remains a matter of debate. The objective of this study was to quantify the association between peripheral blood Tregs and disease status in SLE patients. METHOD: EMBASE and PubMed databases were searched using 'systemic lupus erythematosus' and 'regulatory T-cells' as relevant key terms. A meta-analysis of studies that examined the proportion of Tregs among peripheral blood mononuclear cells (PBMCs) and CD4+T-cells was performed using Stata software. Subgroup analysis was performed based on ethnic groups and Treg definition markers. RESULTS: The Treg/PBMC and Treg/CD4+T-cell ratios were significantly lower in SLE patients than in healthy controls (HCs), whereas patients with active and inactive SLE showed no difference in these indicators. A subgroup analysis indicated that Asian SLE patients had a substantially lower proportion of Tregs/PBMCs than HCs, but this difference was not seen for white and Latin American SLE patients. Patients defined by CD4+CD25+Foxp3+, CD4+CD25+ and CD4+Foxp3+ had a much lower Treg/PBMC ratio compared with HCs. Ethnic groups and choice of Treg definition markers had no influence on the proportion of Tregs/CD4+T-cells. CONCLUSION: The proportion of Tregs among both PBMCs and CD4+T-cells was significantly decreased in SLE patients. Ethnic group and Treg definition markers may influence the proportion of Tregs among PBMCs. Further study of the correlation between SLE disease activity and the proportion of Tregs in peripheral blood is needed to determine the physiological role of this association.


Assuntos
Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Linfócitos T Reguladores/imunologia , Adolescente , Adulto , Contagem de Linfócito CD4 , Grupos Étnicos , Feminino , Humanos , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Pessoa de Meia-Idade , Adulto Jovem
6.
Reumatol. clín. (Barc.) ; 15(1): 34-42, ene.-feb. 2019. tab
Artigo em Inglês | IBECS | ID: ibc-176075

RESUMO

Objectives: The course and long-term outcome of pure membranous lupus nephritis (MLN) are little understood. The aims of this study are to evaluate the clinical features, course, outcome and prognostic indicators in pure MLN and to determine the impact of ethnicity and the type of health insurance on the course and prognosis of pure MLN. Methods: We conducted a retrospective review of medical records of 150 patients with pure MLN from Spain and the USA. Results: Mean age was 34.2±12.5 and 80% were women. Sixty-eight percent of patients had nephrotic syndrome at diagnosis. The average serum creatinine was 0.98±0.78mg/dl. Six percent of patients died and 5.3% developed end-stage renal disease (ESRD). ESRD was predicted by male sex, hypertension, dyslipidemia, high basal 24h-proteinuria, high basal serum creatinine and a low basal creatinine clearance. Age, cardiac insufficiency, peripheral artheriopathy, hemodialysis and not having received mycophenolate mofetil or antimalarials for MLN predicted death. Conclusions: Pure MLN frequently presents with nephrotic syndrome, high proteinuria and normal serum creatinine. Its prognosis is favourable in maintaining renal function although proteinuria usually persists over time. Baseline cardiovascular disease and not having a health insurance are related with poor prognosis


Objetivos: Los conocimientos sobre el curso y el desenlace a largo plazo de la nefritis lúpica membranosa (NLM) pura son todavía escasos. El objetivo de este estudio es evaluar las características clínicas, curso, desenlace e indicadores pronósticos de la NLM y determinar el impacto de la etnicidad y tipo de cobertura sanitaria en el curso y pronóstico de la NLM. Métodos: Se realizó una revisión retrospectiva de las historias de 150 pacientes con NLM de España y Estados Unidos. Resultados: La edad media fue 34,2±12,5 y el 80% eran mujeres. El 68% de los pacientes tenían síndrome nefrótico al diagnóstico. La creatinina sérica media fue 0,98±0,78mg/dl. El 6% de los pacientes fallecieron y el 5,3% desarrollaron insuficiencia renal terminal (IRT). El sexo masculino, la hipertensión, la dislipemia, la alta proteinuria basal, la alta creatininemia y un aclaramiento de creatinina reducido predijeron el desarrollo de IRT. La edad, la insuficiencia cardíaca, la arteriopatía periférica, la hemodiálisis y el no haber recibido micofenolato de mofetilo o antimaláricos predijeron el fallecimiento. Conclusiones: La NLM pura suele debutar con síndrome nefrótico, alta proteinuria y creatininemia normal. Su pronóstico es favourable en términos de mantenimiento de la función renal aunque la proteinuria habitualmente persiste durante el seguimiento. La enfermedad cardiovascular basal y no tener cobertura sanitaria se relacionan con mal pronóstico


Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Nefrite Lúpica/epidemiologia , Glomerulonefrite Membranosa/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Estudos Retrospectivos , Proteinúria/epidemiologia , Avaliação de Resultados da Assistência ao Paciente , Lúpus Eritematoso Sistêmico/etnologia , Creatinina/sangue
7.
Arthritis Care Res (Hoboken) ; 71(1): 95-103, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29669194

RESUMO

OBJECTIVE: Relative to studies of systemic lupus erythematosus (SLE), epidemiologic studies of chronic cutaneous lupus erythematosus (CCLE) are rare and are limited to populations with no racial diversity. We sought to provide minimum estimates of the incidence of primary CCLE (CCLE in the absence of SLE) in a population comprised predominantly of white individuals and black individuals in the southeastern region of the US. METHODS: The Georgia Lupus Registry allowed for the use of multiple sources for case-finding, including dermatology and rheumatology practices, multispecialty health care facilities, and dermatopathology reports. Cases with a clinical or clinical/histologic diagnosis of CCLE were classified as definite. Cases ascertained exclusively from dermatopathology reports were categorized as probable. Age-standardized incidence rates stratified by sex and race were calculated for discoid lupus erythematosus (DLE) in particular and for CCLE in general. RESULTS: The overall age-adjusted estimates for combined (definite and probable) CCLE were 3.9 per 100,000 person-years (95% confidence interval [95% CI] 3.4-4.5). The overall age-adjusted incidences of definite and combined DLE were 2.9 (95% CI 2.4-3.4) and 3.7 (95% CI 3.2-4.3) per 100,000 person-years, respectively. When capture-recapture methods were used, the age-adjusted incidence of definite DLE increased to 4.0 (95% CI 3.2-4.3). The black:white and female:male incidence ratios for definite DLE were 5.4 and 3.1, respectively. CONCLUSION: Our findings underscore the striking racial disparities in susceptibility to primary CCLE, with black individuals having a 3-fold to 5-fold increased incidence of CCLE in general, and DLE in particular, compared with white individuals. The observed sex differences were consistent with those reported previously, with a 3 times higher risk of DLE in women compared with men.


Assuntos
Afro-Americanos/etnologia , Grupo com Ancestrais do Continente Europeu/etnologia , Disparidades em Assistência à Saúde/etnologia , Lúpus Eritematoso Discoide/etnologia , Lúpus Eritematoso Sistêmico/etnologia , Sistema de Registros , Adulto , Feminino , Georgia/etnologia , Humanos , Incidência , Lúpus Eritematoso Discoide/diagnóstico , Lúpus Eritematoso Discoide/terapia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Sudeste dos Estados Unidos/etnologia
8.
Lupus ; 28(2): 253-260, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30482093

RESUMO

OBJECTIVES: Systemic lupus erythematosus (SLE) disproportionately strikes African American women. Social support can potentially reduce disease impact. The purpose of this study is to understand the relationship between organ damage and depression in African American women and how social support influences this relationship. METHODS: We used a mixed methods design, analyzing self-reported data on lupus-related organ damage, depression, and social support in 437 African American women with SLE recruited in the Georgians Organized Against Lupus (GOAL) cohort. Moreover, we conducted interviews among 15 GOAL participants to gather patients' perspectives about the role of social support in people who live with lupus. RESULTS: We found a significant association between organ damage and depression ( r = 0.163, p = 0.001), as well as between depression and social support ( F = 17.574, p < 0.001). The quantitative analysis did not render social support as a significant moderator in the organ damage-depression relationship. Interviews, however, revealed that African American women with the most severe organ damage have the greatest need for support. CONCLUSIONS: Social support is a key resource for lupus patients with high disease burden. Overall, these findings highlight the importance of monitoring depressive symptoms in this population and developing interventions aimed to increase social support available to lupus patients.


Assuntos
Afro-Americanos/psicologia , Depressão/psicologia , Lúpus Eritematoso Sistêmico/psicologia , Apoio Social , Adulto , Afro-Americanos/estatística & dados numéricos , Estudos de Coortes , Depressão/etnologia , Feminino , Georgia , Humanos , Entrevistas como Assunto , Modelos Lineares , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Pessoa de Meia-Idade , Escalas de Graduação Psiquiátrica , Autorrelato , Índice de Gravidade de Doença
9.
Semin Arthritis Rheum ; 48(5): 840-846, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30205982

RESUMO

OBJECTIVE: Systemic lupus erythematosus (SLE), which is associated with increased stroke risk, is more prevalent and often more severe among Blacks, Asians, and Hispanics than Whites. We examined racial/ethnic variation in stroke rates and risks, overall and by hemorrhagic versus ischemic subtype, among SLE patients. METHODS: Within Medicaid (2000-2010), we identified patients aged 18-65 with SLE (≥ 3 ICD-9 710.0 codes, ≥ 30days apart) and ≥12 months of continuous enrollment. Subjects were followed from index date to first stroke event, death, disenrollment, or end of follow-up. Race/ethnicity-specific annual event rates were calculated for stroke overall and by subtypes (hemorrhagic vs. ischemic). We used Cox proportional hazard models to estimate hazard ratios (HR) of stroke by race/ethnicity, adjusting for comorbidities and the competing risk of death. RESULTS: Of 65,788 SLE patients, 93.1% were female. Racial/ethnic breakdown was 42% Black, 38% White, 16% Hispanic, 3% Asian, and 1% American Indian/Alaska Natives. Mean follow-up was 3.7 ± 3.0years. After multivariable adjustment, Blacks were at increased risk of overall stroke (HR 1.34 [95%CI 1.18-1.53), hemorrhagic stroke (HR 1.42 [1.00-2.01]), and ischemic stroke (HR 1.33 [1.15-1.52]) compared to Whites. Hispanics were at increased risk of overall stroke (HR 1.25 [1.06-1.47)] and hemorrhagic stroke (HR 1.79 [95% CI 1.22-2.61]), but not ischemic stroke, compared to Whites. CONCLUSION: Among SLE patients enrolled in Medicaid, we observed elevated stroke risk (overall and by subtype) among Blacks and Hispanics compared to Whites, suggesting the importance of early recognition and screening for stroke risk factors among Blacks and Hispanics.


Assuntos
Lúpus Eritematoso Sistêmico/etnologia , Acidente Vascular Cerebral/etnologia , Adulto , Afro-Americanos/estatística & dados numéricos , Idoso , Americanos Asiáticos/estatística & dados numéricos , Estudos de Coortes , Comorbidade , Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Feminino , Hispano-Americanos/estatística & dados numéricos , Humanos , Índios Norte-Americanos/estatística & dados numéricos , Masculino , Medicaid/estatística & dados numéricos , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estados Unidos/epidemiologia , Adulto Jovem
10.
Expert Rev Clin Immunol ; 14(12): 1043-1053, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30338717

RESUMO

INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by organ damage, flare-remission pattern, and increased mortality when compared with the general population. SLE mortality depends on epidemiological, sociodemographic, genetic, and clinical factors. Mortality causes have been mainly grouped in disease activity, infections, and cardiovascular complications. Lupus nephritis and neuropsychiatric lupus are the main manifestations associated to mortality. Bacterial infection remains an important cause of death, and cardiovascular mortality is almost double when compared to age - and sex-matched comparisons. Characteristics such as time from onset to diagnosis > 1-year, renal involvement, high SLEDAI and severe organ involvement, may be predictors of mortality. Interventions including steroids, immunosuppressants, plasmapheresis, some biologics, and vaccination have shown efficacy in reducing mortality rates. Areas covered: In this narrative review the epidemiology, main causes of mortality, potential predictors, and interventions are described. Expert commentary: Despite early diagnosis and immunosuppressive treatment, SLE mortality remains high. African-American, Hispanic-American origin, low socioeconomic status and male sex are associated with increased mortality. Currently, there is no unique, precise prediction model for mortality; however, predictors for increased activity such as infections and cardiovascular events, lead to increased mortality. New prediction models may indicate early interventions in order to improve mortality rates.


Assuntos
Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/terapia , Afro-Americanos , Causas de Morte , Progressão da Doença , Hispano-Americanos , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/etnologia , Medição de Risco , Fatores de Risco , Fatores Sexuais , Determinantes Sociais da Saúde , Fatores Socioeconômicos , Fatores de Tempo , Resultado do Tratamento , Estados Unidos/epidemiologia
11.
Pediatr Rheumatol Online J ; 16(1): 56, 2018 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-30201026

RESUMO

BACKGROUND: Accumulated damage is an important prognostic factor in systemic lupus erythematous. However, the pattern of disease damage and its risk factors have not been well studied in childhood-onset systemic lupus erythematosus (cSLE) in Asia. The objectives are to evaluate the pattern of damage and to identify the risk factors for accumulated damage in an Asian group of cSLE. METHODS: A retrospective chart review was conducted on a group of 59 patients with cSLE. Patient demographics and clinical variables were first collected at diagnosis. Over the course of their disease, clinical variables considered as risk factors for damage were also collected. Damage was measured using the Systemic Lupus International Collaborating Clinics/ American College of Rheumatology Damage Index (SDI) for each patient at their last encounter. Based on their SDI scores, patients were then dichotomized to two groups: a group with presence of disease damage (SDI ≥1) and a group with absence of disease damage (SDI score = 0). Clinical variables including age at diagnosis, gender, ethnicity, disease duration, disease manifestations, laboratory values at diagnosis, disease activity at diagnosis and last encounter, major organ involvement, number of lupus flares, major infection, and intensity of immunosuppressive medications were compared between the two groups. Growth failure and estimated glomerular filtration rate (eGFR) were also analysed as secondary outcomes. RESULTS: After a median disease duration and follow up of 7.8 years, 39 patients (66.1%) had no disease damage while 20 patients (33.9%) had acquired disease damage. Disease damage most frequently occurred in the ocular (15.3%), neuropsychiatric (11.9%) and musculoskeletal (11.9%) domains. The most frequent forms of damage were cataracts (11.9%), and avascular necrosis (unilateral and bilateral combined 10.2%). After controlling for other variables, presence of neuropsychiatric manifestations remained the only statistically significant risk factor for damage. The rate of growth failure in our group of patients was 16%. Patients who experienced growth failure were significantly younger at disease diagnosis. The median age of diagnosis was 10 for those who experienced growth failure, whereas the median age of diagnosis was 13 for those who did not experience growth failure. Despite a high rate of renal involvement in the group (79.7%), renal damage was only seen in 3.2% of the patients. 91.5% of the studied group had normal eGFR of ≥90 ml/min/1.73m2 at their last follow up. CONCLUSION: This group of patients had a low rate of damage accrual, with one of the lowest rates in renal damage when compared to other cohorts reported. The presence of neuropsychiatric manifestations was identified as the most significant risk factor for disease damage, while the most frequent forms of damage were cataracts and avascular necrosis, which were both related to prolonged steroid use. Despite the limitations of this study, it highlights the need for larger prospective studies to understand the relationship between childhood-onset SLE and its resulting damage.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Adolescente , Grupo com Ancestrais do Continente Asiático , Estudos de Casos e Controles , Criança , Progressão da Doença , Feminino , Seguimentos , Taxa de Filtração Glomerular , Transtornos do Crescimento/epidemiologia , Transtornos do Crescimento/etiologia , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença
12.
Yonsei Med J ; 59(7): 857-864, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30091319

RESUMO

PURPOSE: The aim of this study was to identify the associations among physical activity, disease activity, and organ damage in patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: A total of 415 patients with SLE were consecutively enrolled from the KORean lupus Network (KORNET) registry. This registry assessed clinical features, disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)], and organ damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)] upon enrollment in the study. Self-reported physical activity was measured by the International Physical Activity Questionnaire. Statistical analyses were conducted using the Mann-Whitney U test and multivariate logistic regression analysis. RESULTS: A significant difference in vigorous activity was noted between patients with lupus nephritis (LN) (n=93) and those without LN (n=322) (p=0.012), but not in moderate and walking activities. In contrast, no differences in physical activity, walking, moderate, and vigorous intensity, according to SLEDAI-2K and SDI were found. In addition to younger age (p=0.032), high physical component summary of SF-36 (p=0.004) and SLEDAI-2K (p=0.038), and less vigorous physical activity were associated with LN (p=0.024). However, cardiovascular disease was not associated with physical activity in SLE patients. CONCLUSION: This study showed that patients with LN had less vigorous physical activity than patients without LN. The results suggest that lupus nephritis might be associated with physical activity.


Assuntos
Exercício , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Nefrite Lúpica/etnologia , Adulto , Estudos Transversais , Progressão da Doença , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Nefrite Lúpica/complicações , Masculino , Pessoa de Meia-Idade , Sistema de Registros , República da Coreia/epidemiologia , Autorrelato , Índice de Gravidade de Doença
13.
Lupus ; 27(12): 1989-1995, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30092732

RESUMO

Objectives Hispanics with systemic lupus erythematosus (SLE) in the United States have more severe disease and damage accrual compared with whites. Data on Hispanics of similar ancestry in geographically different locations is limited but essential in defining genetic and environmental factors for SLE. This study evaluates SLE disease burden in two Dominican communities, Washington Heights in New York City (NYC) and Santiago in the Dominican Republic (DR). Methods Disease activity (SLE Disease Activity Index 2000 (SLEDAI-2K)) and damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) were cross-sectionally measured in 76 Dominican SLE patients from the Columbia University Lupus Cohort in NYC and compared with 75 Dominican SLE patients living in Santiago in the DR. Results Mean (±SD) age was 40 (±14) and 36 (±11) years for NYC and DR patients, respectively. Median disease duration was 8 years. Disease activity was mild in both groups (SLEDAI-2K of 3 in NYC versus 4 in the DR). NYC Dominicans had more discoid lesions, positive anti-dsDNA, and anti-SSB antibodies. Dominicans in the DR used more corticosteroids, had less medical insurance, lower educational level, and were more likely to be unemployed, whereas more Dominicans in NYC smoked. NYC patients had a higher SDI compared with SLE patients in the DR (0.96 versus 0.24, p < 0.0001). Statistical significance was maintained in adjusted analysis (1.26 versus 0.57, p < 0.0001). Conclusion SLE Dominican patients in NYC had a higher SDI than those in the DR. Longitudinal studies are needed to ascertain whether this difference is due to biological, environmental factors, immigration patterns or a survival bias.


Assuntos
Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Índice de Gravidade de Doença , Corticosteroides/uso terapêutico , Adulto , Progressão da Doença , República Dominicana , Feminino , Hispano-Americanos , Humanos , Modelos Lineares , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Cidade de Nova Iorque/etnologia
14.
Mol Med ; 24(1): 24, 2018 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-30134810

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease with genetic, hormonal, and environmental influences. In Western Europe and North America, individuals of West African descent have a 3-4 fold greater incidence of SLE than Caucasians. Paradoxically, West Africans in sub-Saharan Africa appear to have a low incidence of SLE, and some studies suggest a milder disease with less nephritis. In this study, we analyzed sera from African American female SLE patients and four other cohorts, one with SLE and others with varying degrees of risk for SLE in order to identify serologic factors that might correlate with risk of or protection against SLE. METHODS: Our cohorts included West African women with previous malaria infection assumed to be protected from development of SLE, clinically unaffected sisters of SLE patients with high risk of developing SLE, healthy African American women with intermediate risk, healthy Caucasian women with low risk of developing SLE, and women with a diagnosis of SLE. We developed a lupus risk index (LRI) based on titers of IgM and IgG anti-double stranded DNA antibodies and levels of C1q. RESULTS: The risk index was highest in SLE patients; second highest in unaffected sisters of SLE patients; third highest in healthy African-American women and lowest in healthy Caucasian women and malaria-exposed West African women. CONCLUSION: This risk index may be useful in early interventions to prevent SLE. In addition, it suggests new therapeutic approaches for the treatment of SLE.


Assuntos
Predisposição Genética para Doença , Lúpus Eritematoso Sistêmico , Adolescente , Adulto , Grupo com Ancestrais do Continente Africano , Idoso , Anticorpos Antinucleares/sangue , Complemento C1q/análise , Grupo com Ancestrais do Continente Europeu , Feminino , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/genética , Lúpus Eritematoso Sistêmico/imunologia , Malária/sangue , Malária/etnologia , Malária/genética , Malária/imunologia , Pessoa de Meia-Idade , Adulto Jovem
15.
Lupus ; 27(10): 1742-1752, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30060721

RESUMO

Objectives To investigate the mortality and causes of death in Chinese patients with systemic lupus erythematosus. Methods We collected the clinical data of all consecutive adult systemic lupus erythematosus patients at the Rheumatology department of Peking University First Hospital between January 2007 and December 2015. The primary causes of death were identified, the standardized mortality ratio and years of life lost were calculated, and the survival and variables associated with mortality were determined by Kaplan-Meier and Cox regression analysis respectively. Results The mean age of all 911 patients (814 females and 97 males) was 37.8 ± 14.7 years, the median disease duration at recruitment was 2.6 (0.5-7.0) years, and the median follow-up duration was 3.0 (1.4-5.1) years. Among the 911 patients who were successfully followed up, 45 patients died. Infection (31.1%) was the leading cause of death followed by renal failure, pulmonary arterial hypertension and cerebrovascular diseases. The overall age and sex-adjusted standardized mortality ratio was 3.2 (95% confidence interval 2.4-4.0), and the years of life lost for women and men were 29.8 and 9.4 respectively. Overall survival at 1, 5 and 10 years was 98.2%, 95.3% and 93.7% respectively. Older age at disease onset, infection, autoimmune hemolytic anemia, thrombocytopenia and pulmonary arterial hypertension were independent risk factors for the mortality of systemic lupus erythematosus patients, and longer disease duration at recruitment was an independent protective factor. Conclusions Mortality of systemic lupus erythematosus patients in China was substantial, especially in females, with infection the leading cause of death. Older age at disease onset, infection, autoimmune hemolytic anemia, thrombocytopenia and pulmonary arterial hypertension were associated with poor outcomes.


Assuntos
Lúpus Eritematoso Sistêmico/mortalidade , Adulto , Distribuição por Idade , Fatores Etários , Grupo com Ancestrais do Continente Asiático , Causas de Morte , China/epidemiologia , Comorbidade , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Distribuição por Sexo , Fatores Sexuais , Fatores de Tempo , Adulto Jovem
16.
Arthritis Res Ther ; 20(1): 193, 2018 08 29.
Artigo em Inglês | MEDLINE | ID: mdl-30157968

RESUMO

BACKGROUND: Type III interferons (IFNs) or IFN-λs are the newly discovered cytokines that primarily target the cells of epithelial and myeloid lineages, which are major components of kidneys. The current study aimed to investigate whether IFN-λs are involved in the pathogenesis of systemic lupus erythematosus (SLE) and lupus nephritis. METHODS: TaqMan allele discrimination assays were used to determine IFNL3/4 SNP genotypes of 1620 healthy controls and 1013 SLE patients (two independent cohorts consisting of 831 and 182 subjects, respectively) from Taiwan. The distributions of IFNL3/4 SNP genotypes and allele frequencies were compared between SLE patients and healthy controls and among SLE patients stratified by clinical phenotypes. ELISA was used to determine the serum IFN-λ3 concentrations of SLE patients. RESULTS: All major IFN3/4 SNP alleles were significantly associated with the risk for lupus nephritis (rs8099917T, PFDR = 0.0021, OR 1.75, 95% CI 1.24-2.47; rs12979860C, PFDR = 0.0034, OR 1.65, 95% CI 1.18-2.30; rs4803217C, PFDR = 0.0021, OR 1.76, 95% CI 1.25-2.48; and ss469415590TT, PFDR = 0.0021, OR 1.73, 95% CI 1.23-2.42) among SLE patients. Similarly, the major IFNL3/4 SNP haplotype rs8099917T-ss469415590TT-rs12979860C-rs4803217C (or T-TT-C-C) was a significant risk factor for lupus nephritis (P = 0.0015, OR 1.68, 95% CI 1.22-2.32). Additionally, all minor IFN3/4 SNP alleles were significantly associated with SLE susceptibility in nephritis-negative SLE patients as compared to normal healthy controls (rs8099917G, PFDR = 0.00177, OR 1.68, 95% CI 1.24-2.28; rs12979860T, PFDR = 0.00299, OR 1.58, 95% CI 1.18-2.32; rs4803217A, PFDR = 0.00176, OR 1.65, 95% CI 1.22-2.23; and ss469415590ΔG, PFDR = 0.00176, OR 1.70, 95% CI 1.26-2.29). Furthermore, the elevated serum levels of IFN-λ3 were significantly correlated with the complement depression and the high SLE disease activities in SLE patients. CONCLUSIONS: IFN-λ3/4 genetic variants play a unique role in the development of lupus nephritis and SLE.


Assuntos
Predisposição Genética para Doença/genética , Interleucinas/genética , Nefrite Lúpica/genética , Polimorfismo de Nucleotídeo Único , Adulto , Grupo com Ancestrais do Continente Asiático/genética , Biomarcadores/sangue , Feminino , Frequência do Gene , Predisposição Genética para Doença/etnologia , Genótipo , Haplótipos , Humanos , Interferons , Interleucinas/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/genética , Nefrite Lúpica/sangue , Nefrite Lúpica/etnologia , Masculino , Pessoa de Meia-Idade , Taiwan , Adulto Jovem
17.
Lupus ; 27(10): 1735-1741, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30045666

RESUMO

Introduction The Systemic Lupus Erythematosus Activity Questionnaire (SLAQ) is a patient-reported instrument for the assessment of disease activity in systemic lupus erythematosus (SLE). The aims of the present study are translation, cultural adaptation and validation of an Italian version: the SLAQit. Methods The process of translation and cultural adaptation followed published guidelines. SLAQit was pretested in a group of 35 SLE patients to evaluate acceptability, comprehension and feasibility. Internal consistency, test-retest validity and external validity were tested on consecutive SLE patients attending the clinic. Results In total, 135 SLE patients were enrolled in this study. The pilot test provided a 99.9% response rate and demonstrated feasibility and comprehensibility of the questionnaire. A good internal consistency was found among the three components of the score (SLAQ score, numerical rating scale (NRS), patient global assessment question (PGA); α = 0.79). SLAQit showed very high reliability (test-retest α > 0.8). NRS and PGA showed a strong positive correlation with both Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) ( p = 0.002 and p < 0.001, respectively) and European Consensus Lupus Measurement (ECLAM) scores ( p = 0.01 and p < 0.001, respectively), while the SLAQ score did not. A significant agreement was observed between the physician's intention to treat and both the NRS and PGA scores, while no significant association was reported with the SLAQ score. Conclusions SLAQit was demonstrated to be a reliable and valid instrument for self-assessment of disease activity in SLE patients.


Assuntos
Características Culturais , Grupo com Ancestrais do Continente Europeu/psicologia , Conhecimentos, Atitudes e Prática em Saúde/etnologia , Lúpus Eritematoso Sistêmico/diagnóstico , Medidas de Resultados Relatados pelo Paciente , Tradução , Adulto , Compreensão , Estudos de Viabilidade , Feminino , Humanos , Itália/epidemiologia , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/psicologia , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença
18.
Arthritis Res Ther ; 20(1): 152, 2018 07 27.
Artigo em Inglês | MEDLINE | ID: mdl-30053827

RESUMO

BACKGROUND: The molecular heterogeneity of autoimmune and inflammatory diseases has been one of the main obstacles to the development of safe and specific therapeutic options. Here, we evaluated the diagnostic and clinical value of a robust, inexpensive, immunoassay detecting the circulating soluble form of the monocyte-specific surface receptor sialic acid binding Ig-like lectin 1 (sSIGLEC-1). METHODS: We developed an immunoassay to measure sSIGLEC-1 in small volumes of plasma/serum from systemic lupus erythematosus (SLE) patients (n = 75) and healthy donors (n = 504). Samples from systemic sclerosis patients (n = 99) were studied as an autoimmune control. We investigated the correlation between sSIGLEC-1 and both monocyte surface SIGLEC-1 and type I interferon-regulated gene (IRG) expression. Associations of sSIGLEC-1 with clinical features were evaluated in an independent cohort of SLE patients (n = 656). RESULTS: Plasma concentrations of sSIGLEC-1 strongly correlated with expression of SIGLEC-1 on the surface of blood monocytes and with IRG expression in SLE patients. We found ancestry-related differences in sSIGLEC-1 concentrations in SLE patients, with patients of non-European ancestry showing higher levels compared to patients of European ancestry. Higher sSIGLEC-1 concentrations were associated with lower serum complement component 3 and increased frequency of renal complications in European patients, but not with the SLE Disease Activity Index clinical score. CONCLUSIONS: Our sSIGLEC-1 immunoassay provides a specific and easily assayed marker for monocyte-macrophage activation, and interferonopathy in SLE and other diseases. Further studies can extend its clinical associations and its potential use to stratify patients and as a secondary endpoint in clinical trials.


Assuntos
Biomarcadores/sangue , Interferon-alfa/biossíntese , Lúpus Eritematoso Sistêmico/sangue , Lectina 1 Semelhante a Ig de Ligação ao Ácido Siálico/sangue , Adulto , Idoso , Feminino , Humanos , Imunoensaio/métodos , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/imunologia , Nefrite Lúpica/sangue , Nefrite Lúpica/etnologia , Nefrite Lúpica/imunologia , Masculino , Pessoa de Meia-Idade , Transcriptoma , Adulto Jovem
19.
Lupus ; 27(10): 1577-1581, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30016928

RESUMO

Despite a marked improvement in 10-year survival for systemic lupus erythematosus (SLE) patients over the past five decades, mortality rates from SLE remain high compared to those in the general population. SLE was also among the leading causes of death in young women in the United States during 2000-2015. However, it is encouraging that SLE mortality rates and the ratios of SLE mortality rates to non-SLE mortality rates have decreased every year since the late 1990s. Despite this improvement, disparities in SLE mortality persist according to sex, race, age, and place of residence. Furthermore, demographic and geographic variables seem to modify the effect of each other in influencing SLE mortality, leading to interactions between sex/race/ethnicity-associated factors and geographic differences. In other words, individuals of the same sex/race/ethnicity had differences in SLE mortality depending on where they lived. These observations highlight SLE as an important public health issue. The recognition of SLE as a leading cause of death in the general population might spur targeted public health programs and research funding to address the high lupus mortality.


Assuntos
Saúde Global/tendências , Lúpus Eritematoso Sistêmico/mortalidade , Distribuição por Idade , Anti-Inflamatórios/uso terapêutico , Causas de Morte , Feminino , Humanos , Imunossupressores/uso terapêutico , Incidência , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/etnologia , Masculino , Mortalidade/tendências , Prognóstico , Fatores de Risco , Distribuição por Sexo , Fatores de Tempo
20.
Gene ; 668: 59-72, 2018 Aug 20.
Artigo em Inglês | MEDLINE | ID: mdl-29775752

RESUMO

Systemic lupus erythematosus (SLE; OMIM 152700) is a highly heterogeneous disorder, characterized by differences in autoantibody profile, serum cytokines, and a multi-system involvement commonly affecting the skin, renal, musculoskeletal, and hematopoetic systems clinical manifestations involving. Disease features range from mild manifestations, such as rash or arthritis, to life-threatening end-organ manifestations, such as glomerulonephritis or thrombosis, and it is difficult to predict which manifestations will affect a given patient. SLE is caused by interactions between susceptibility genes and environmental factors resulting in an irreversible loss of immunologic self-tolerance. Incidence is highest in women during the reproductive years; however, people of all ages, genders, and ancestral backgrounds are susceptible. A striking 9:1 female to male differential appears in incidence, which remains largely unexplained. However, people of both sexes and all ages and ethnic backgrounds are susceptible. Distinct differences regarding the pathogenesis of SLE between patients of different ancestral backgrounds have been observed so far, including differences in specific clinical manifestations, disease-susceptibility genetic variants and IFN levels. Genome-wide association studies (GWAS) have attempted to elucidate partially the complex genetic architecture of SLE and to point out the existing differences in risk variants across different continental populations, considering that some alleles have not been found in all ancestral backgrounds. Levels of circulating IFN-α is a heritable risk factor in SLE with causal role in pathogenesis, they differ between SLE patients from different ancestral backgrounds and this information could be important as therapeutics is developed to target this pathway. This review highlights some recent findings referred to the multilevel differences appearing in SLE patients from different ancestral backgrounds and further understanding of this knowledge may permit the development of personalized treatments based on patients' ancestry.


Assuntos
Predisposição Genética para Doença , Lúpus Eritematoso Sistêmico/genética , Grupos de Populações Continentais , Estudos de Associação Genética , Loci Gênicos , Humanos , Interferons/metabolismo , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/metabolismo , Polimorfismo de Nucleotídeo Único
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