RESUMO

OBJECTIVE: To evaluate the progression of coronary artery calcification (CAC) and associated risk factors in a systemic lupus erythematosus (SLE) cohort. METHODS: We reassessed the presence of CAC in patients with SLE who were screened 9 years before, using multidetector computed tomography. Clinical variables (cumulated disease activity and damage accrual), antiphospholipid syndrome and SLE serology, and cardiovascular (CV) risk factors (hypertension, BMI [kg/m2], modified Framingham risk score, lipid profile, menopausal status) were assessed longitudinally. RESULTS: We included 104 patients from the parent study. Most of them were women (94.2%), with a mean age of 41.0 (SD 8.3) years and mean disease duration of 14.8 (SD 2.9) years. We documented CAC in 17 patients (16.3%). Seven cases were from the parent study and 10 were incident cases. The cumulative incidence of CAC was 9% and the incidence density was 1 per 100 person-years. CAC occurred more frequently in the age groups 30-39 years and 40-44 years. All patients with previous CAC had worsening of their calcium indexes, and none developed clinical CV events. When comparing prevalent CAC cases (n = 17) vs patients without calcification (n = 87), both groups were similar in traditional CV risk factors, disease duration, Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) area under the curve (AUC), and Systemic Lupus International Collaborating Clinics (SLICC) score, but were more likely to be postmenopausal and have higher apolipoprotein B (apoB) levels. Patients with previous CAC had higher apoB levels, SLEDAI-2K AUC scores, and anticardiolipin IgG antibodies than incident cases. CONCLUSION: CAC in patients with SLE progressed over time but was not associated with adverse CV events during the first 9 years of follow-up. ApoB levels and postmenopausal status might be associated with this progression.

Assuntos

Doença da Artéria Coronariana , Progressão da Doença , Lúpus Eritematoso Sistêmico , Calcificação Vascular , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/diagnóstico por imagem , Feminino , Adulto , Masculino , Pessoa de Meia-Idade , Doença da Artéria Coronariana/epidemiologia , Doença da Artéria Coronariana/diagnóstico por imagem , Calcificação Vascular/diagnóstico por imagem , Calcificação Vascular/epidemiologia , Fatores de Risco , Incidência , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/patologia , Estudos Longitudinais

RESUMO

OBJECTIVE: To determine the independent impact of different definitions of remission and low disease activity (LDA) on damage accrual. METHODS: Patients with ≥2 annual assessments from a longitudinal multinational inception lupus cohort were studied. Five mutually exclusive disease activity states were defined: remission off-treatment: clinical Systemic Lupus Erythematosus Disease Activity Index (cSLEDAI)-2K=0, without prednisone or immunosuppressants; remission on-treatment: cSLEDAI-2K score=0, prednisone ≤5 mg/day and/or maintenance immunosuppressants; low disease activity Toronto cohort (LDA-TC): cSLEDAI-2K score of ≤2, without prednisone or immunosuppressants; modified lupus low disease activity (mLLDAS): Systemic Lupus Erythematosus Disease Activity Index-2K score of 4 with no activity in major organ/systems, no new disease activity, prednisone ≤7.5 mg/day and/or maintenance immunosuppressants; active: all remaining visits. Only the most stringent definition was used per visit. Antimalarials were allowed in all. The proportion of time that patients were in a specific state at each visit since cohort entry was determined. Damage accrual was ascertained with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Univariable and multivariable generalised estimated equation negative binomial regression models were used. Time-dependent covariates were determined at the same annual visit as the disease activity state but the SDI at the subsequent visit. RESULTS: There were 1652 patients, 1464 (88.6%) female, mean age at diagnosis 34.2 (SD 13.4) years and mean follow-up time of 7.7 (SD 4.8) years. Being in remission off-treatment, remission on-treatment, LDA-TC and mLLDAS (per 25% increase) were each associated with a lower probability of damage accrual (remission off-treatment: incidence rate ratio (IRR)=0.75, 95% CI 0.70 to 0.81; remission on-treatment: IRR=0.68, 95% CI 0.62 to 0.75; LDA: IRR=0.79, 95% CI 0.68 to 0.92; and mLLDAS: IRR=0.76, 95% CI 0.65 to 0.89)). CONCLUSIONS: Remission on-treatment and off-treatment, LDA-TC and mLLDAS were associated with less damage accrual, even adjusting for possible confounders and effect modifiers.

Assuntos

Antimaláricos , Lúpus Eritematoso Sistêmico , Antimaláricos/uso terapêutico , Progressão da Doença , Feminino , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Prednisona/uso terapêutico , Indução de Remissão , Índice de Gravidade de Doença

RESUMO

OBJECTIVE: In systemic lupus erythematosus (SLE), disease activity and glucocorticoid (GC) exposure are known to contribute to irreversible organ damage. We aimed to examine the association between GC exposure and organ damage occurrence. METHODS: We conducted a literature search (PubMed (Medline), Embase and Cochrane January 1966-October 2021). We identified original longitudinal observational studies reporting GC exposure as the proportion of users and/or GC use with dose information as well as the occurrence of new major organ damage as defined in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. Meta-regression analyses were performed. Reviews, case-reports and studies with <5 years of follow-up, <50 patients, different outcomes and special populations were excluded. RESULTS: We selected 49 articles including 16 224 patients, 14 755 (90.9%) female with a mean age and disease duration of 35.1 years and of 37.1 months. The mean follow-up time was 104.9 months. For individual damage items, the average daily GC dose was associated with the occurrence of overall cardiovascular events and with osteoporosis with fractures. A higher average cumulative dose adjusted (or not)/number of follow-up years and a higher proportion of patients on GC were associated with the occurrence of osteonecrosis. CONCLUSIONS: We confirm associations of GC use with three specific damage items. In treating patients with SLE, our aim should be to maximise the efficacy of GC and to minimise their harms.

Assuntos

Glucocorticoides , Lúpus Eritematoso Sistêmico , Feminino , Glucocorticoides/efeitos adversos , Humanos , Incidência , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Estudos Observacionais como Assunto , Análise de Regressão

RESUMO

OBJECTIVES: To evaluate the performance of the 2016 ACR-EULAR classification Sjögren's syndrome (SS) criteria for classifying patients with secondary SS. METHODS: We randomly selected 300 patients with systemic lupus erythematosus, rheumatoid arthritis and scleroderma, as well as 50 with primary SS. SS diagnosis was established by two independent rheumatologists and was based on the combination of symptoms, signs, diagnostic tests and medical chart review. We evaluated the fulfillment of the 2002 AECG, 2012 ACR and 2016 ACR/EULAR criteria, and their performance using as the gold standard the clinical diagnosis. RESULTS: We identified 154 patients with a clinical (definitive/probable) SS diagnosis, 95 patients (61.7%) fulfilled the AECG, 96 patients (62.3%) the ACR and 90 (58.4%) the 2016 ACR/EULAR criteria. Among the subset with definitive SS clinical diagnosis (n=99), 83 patients (83.8%) fulfilled the AECG, 77 (77.7%) the ACR and 79 (79.7%) the 2016 ACR/EULAR criteria. The concordance rate between the clinical diagnosis (definitive/probable) and the AECG, ACR and 2016 ACR/ EULAR criteria was κ=0.58, κ=0.55 and κ=0.60, respectively. The 2016 ACR/EULAR criteria showed the best AUCs results (0.87 definitive/probable diagnosis, 0.90 definitive diagnosis), followed by the AECG (0.82 definitive/probable diagnosis, 0.85 definitive diagnosis) and ACR (0.80 definitive/probable diagnosis, 0.79 definitive diagnosis) criteria. As a sensitivity analysis, the results were similar when excluding patients with primary SS. CONCLUSIONS: Our study provides further evidence that the 2016 ACR/EULAR criteria are applicable in the setting of secondary SS.

Assuntos

Reumatologia , Esclerodermia Localizada , Síndrome de Sjogren , Área Sob a Curva , Humanos , Satisfação Pessoal , Síndrome de Sjogren/diagnóstico

RESUMO

OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.

Assuntos

Síndrome de Sjogren , Estudos de Coortes , Etnicidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Sistema de Registros , Estudos Retrospectivos , Índice de Gravidade de Doença , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/fisiopatologia

RESUMO

To compare the distribution of HLA-A, B, DRB1 and DQB1 alleles among Mexican patients with primary Sjögren Syndrome (pSS), secondary SS (sSS), connective tissue disease (CTD) without (w/o) SS and historical ethnically healthy controls. We included 28 pSS, 30 sSS, 96 CTD w/o SS patients and 234 controls. HLA-A, B, DRB1 and DQB1 were amplified and sequenced using the Allele SEQR Sequenced Based Typing Kits and analyzed on the ABI Prism*3130 DNA Analyzer using the Assign software. Gene frequencies were obtained by direct counting. Contingence tables of 2 × 2 were generated and analyzed by the Mantel-Haenzel χ (2) or Fisher's test (EPIINFO program). We reported odds ratios (OR) and corrected p values. SS patients showed increased frequencies of A*68:01 and DRB1*14:06 alleles when compared to CTD w/o SS (OR 4.43, 95 % CI 1.35-14.14, p = 0.007 and OR 14, 95 % CI 1.68-116, p = 0.001, respectively) and a higher prevalence of DRB1*01:01 (OR 5.9, 95 % CI 2.13-16.56, p = 0.003) and HLA-B*35:01 (OR 3.70, 95 % CI 1.92-7.12, p = 0.004) when compared with controls. pSS patients had a higher frequency of DRB1*14:06 allele than sSS (OR 16, 95 % CI 1.59-390, p = 0.001). Anti-Ro/SSA positivity was associated with B*51:01 (OR 10.11, 95 % CI 1.09-245, p = 0.02) and DRB1*03:01 alleles (OR 4.26, 95 % CI 1.01-18.89, p = 0.029), whereas the A*01:01 allele was associated with anti-La/SSB positivity (OR 4.75, 95 % CI 1.32-16.92, p = 0.003). In our population, the DRB1*14:06 allele was associated with primary and secondary SS implying that both varieties bear a similar immunogenetic background.

Assuntos

Antígenos HLA/genética , Síndrome de Sjogren/genética , Adulto , Idoso , Alelos , Feminino , Frequência do Gene , Humanos , Imunogenética , Masculino , México , Pessoa de Meia-Idade , Síndrome de Sjogren/imunologia

RESUMO

OBJECTIVE: The aim of this study was to estimate the age at natural menopause in women with SLE. METHODS: One thousand and thirty-nine consecutive SLE patients <60 years of age were surveyed. Demographic and clinical data were queried by a single investigator. SLE characteristics and co-morbidities were retrieved from their medical records. Natural menopause was defined as amenorrhoea ≥12 months in the absence of previous hysterectomy, CYC exposure and severe chronic kidney disease (SCKD). Pregnant women and those with menses during the 12 months prior to interview were considered premenopausal. Median age at menopause was estimated by both logit and survival analyses. In addition, mean age at menopause was calculated for patients aged ≥40 years. Factors associated with age at natural menopause were assessed by Cox regression analysis. RESULTS: A total of 961 SLE women were analysed. At interview, most patients (81.6%) were premenopausal, 7.9% had natural menopause, 6.3% were postmenopausal previously exposed to CYC, 4.1% had undergone hysterectomy before menopause and 0.1% presented with SCKD and amenorrhoea. The mean age at interview was 35.2 years (s.d. 10.1), the mean age at SLE diagnosis was 26.9 years (s.d. 8.6) and the mean duration of disease was 8.2 years (s.d. 7.1). The mean recalled age at menopause was 46.4 years (s.d. 4.7). Median age at menopause estimated by logit and survival analyses were 50.7 and 50.8 years, respectively. Only the age at SLE diagnosis was associated with age at natural menopause. CONCLUSION: Median age at natural menopause in women with lupus is 50 years. This is consistent with the age at menopause reported in the general population.

Assuntos

Lúpus Eritematoso Sistêmico/epidemiologia , Menopausa , Medição de Risco/métodos , Saúde da Mulher , Adulto , Distribuição por Idade , Fatores Etários , Feminino , Seguimentos , Humanos , México/epidemiologia , Pessoa de Meia-Idade , Fatores de Risco , Taxa de Sobrevida/tendências

RESUMO

OBJECTIVE: To determine the prevalence of SS in a cohort of recent-onset SLE patients and evaluate the clinical and immunological variables that may identify SLE patients prone to develop SS. METHODS: A total of 103 patients participating in a prospective cohort of recent-onset SLE were assessed for fulfilment of the American European Consensus Group criteria for SS using a three-phase approach: screening (European questionnaire, Schirmer-I test and wafer test), confirmation (fluorescein staining test, non-stimulated whole-salivary flow and anti-Ro/La antibodies) and lip biopsy. Anti-Ro/SSA and anti-La/SSB antibodies and RF were measured at entry into the cohort and at SS assessment. RESULTS: Ninety-three females and 10 males were included. Mean age at lupus diagnosis was 25.9 ± 8.9 years, and lupus duration at SS assessment was 30.9 ± 9.1 years. SS was diagnosed in 19 (18.5%) patients, all female, and the patients were older at SLE diagnosis than patients without SS (30.8 ± 9.3 vs 24 ± 8.8 years, P = 0.004). Anti-Ro/SSA antibody was more common in SLE-SS patients (84% vs 55%, P = 0.02, LR + 1.53, 95% CI 1.14, 2.04). In the multivariate analysis, age ≥25 years and anti-Ro/SSA antibodies at SLE diagnosis were identified as predictors of SLE-SS, while the absence of anti-Ro/SSA, anti-La/SSB and RF seems to be protective (LR- 0.14, 95% CI 0.02, 0.95). CONCLUSION: The overlap of SLE and SS occurs in almost one-fifth of SLE patients and presents early during its evolution. SLE onset at age ≥25 years plus the presence of anti-Ro/SSA antibody at diagnosis are useful predictors, while the absence of anti-Ro/SSA, anti-La/SSB and RF identifies patients at lowest risk.

Assuntos

Anticorpos Antinucleares/imunologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/epidemiologia , Adulto , Idade de Início , Estudos de Coortes , Intervalos de Confiança , Diagnóstico Precoce , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Análise Multivariada , Valor Preditivo dos Testes , Prevalência , Estudos Prospectivos , Medição de Risco , Síndrome de Sjogren/imunologia , Adulto Jovem

RESUMO

OBJECTIVE: To assess the utility of interferon-α (IFN-α) in serum and cerebrospinal fluid (CSF) as a biomarker of disease activity in central neuropsychiatric systemic lupus erythematosus (cNPSLE). METHODS: Serum and CSF samples were drawn at hospitalization in 34 patients with cNPSLE, 16 surgical SLE, 4 primary neuropsychiatric conditions, and 25 with nonautoimmune conditions, except in 44 non-NPSLE patients in whom only serum was studied. Six months later, serum/CSF and serum samples were taken in 20 cNPSLE and 35 non-NPSLE patients, respectively. SLE activity was assessed at hospitalization, and 6 months later in cNPSLE and non-NPSLE patients. IFN-α was detected by Luminex technology. RESULTS: The mean ± SD age of patients with cNPSLE was 31.4 ± 12.2 years, which was similar across the study groups (p = 0.46). Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores among cNPSLE, non-NPSLE, and SLE-surgical patients were 15.3 ± 8.2, 12.4 ± 8.2, and 3.8 ± 1.5, respectively. IFN-α levels in serum were higher in cNPSLE than in nonautoimmune patients (p = 0.02), but were similar to non-NPSLE and SLE-surgical groups. In CSF samples, IFN-α levels were higher in cNPSLE than in nonautoimmune patients (p = 0.03), and were nonsignificantly higher than in SLE-surgical and primary neuropsychiatric patients. Six months later, serum levels of IFN-α did not vary from baseline values despite a significant decrease in SLEDAI-2K score in cNPSLE and non-NPSLE patients. IFN-α levels in the CSF of patients with cNPSLE also remained stable. Among specific cNPSLE syndromes, CSF IFN-α levels were significantly higher among patients with acute confusional syndrome. CONCLUSION: IFN-α does not seem to represent a useful biomarker of cNPSLE syndromes; its utility in specific cNPSLE manifestations merits further investigation.

Assuntos

Interferon-alfa/sangue , Interferon-alfa/líquido cefalorraquidiano , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Índice de Gravidade de Doença , Adulto , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Estudos de Casos e Controles , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/líquido cefalorraquidiano , Lúpus Eritematoso Sistêmico/diagnóstico , Vasculite Associada ao Lúpus do Sistema Nervoso Central/sangue , Vasculite Associada ao Lúpus do Sistema Nervoso Central/líquido cefalorraquidiano , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes

RESUMO

OBJECTIVE: To define the effects of continuous sequential estrogen plus progestin therapy on menopausal symptoms in women with systemic lupus erythematosus (SLE). METHODS: We performed a randomized, double-blind, 24-month clinical trial involving 106 women with SLE who were in the menopausal transition or early or late postmenopause. Patients received continuous sequential estrogen plus progestin (n = 52) or placebo (n = 54). Menopausal symptoms were assessed using the Greene Climacteric Scale at 0, 1, 2, 3, 6, 9, 12, 15, 18, 21, and 24 months. A new factor analysis of the scale reduced 21 items to 5 factors. The primary outcome was improvement of menopausal symptoms throughout the followup period. Results were analyzed by the intent-to-treat principle. RESULTS: At baseline, demographic and disease characteristics were similar in both groups. Fifteen of 21 menopausal symptoms had a prevalence of ≥50%, with a similar distribution between groups. Vasomotor factor scores decreased over time in both groups (P = 0.002), but in the estrogen plus progestin group the reduction was more pronounced than in the placebo group (1.5-2.0 versus 0.35-0.8 points on a scale of 0-6; P = 0.03). Maximum effects were observed among the most symptomatic women. Psychological, subjective-somatic, and organic-somatic factors scores also improved along time (P < 0.001), but the treatment and placebo arms improved to a similar degree. Thromboses occurred in 3 patients receiving estrogen plus progestin and in 1 patient receiving placebo. CONCLUSION: Menopausal symptoms are highly prevalent in peri- and postmenopausal lupus patients. Estrogen plus progestin improved vasomotor symptoms at a clinically significant level, but not other menopausal symptoms. Given the thrombotic risks of menopausal hormone therapy, this should be used only in women with significant vasomotor symptoms.

Assuntos

Terapia de Reposição de Estrogênios , Estrogênios Conjugados (USP)/administração & dosagem , Lúpus Eritematoso Sistêmico/complicações , Acetato de Medroxiprogesterona/administração & dosagem , Menopausa/efeitos dos fármacos , Progestinas/administração & dosagem , Sistema Vasomotor/efeitos dos fármacos , Adulto , Idoso , Análise de Variância , Distribuição de Qui-Quadrado , Método Duplo-Cego , Terapia de Reposição de Estrogênios/efeitos adversos , Estrogênios Conjugados (USP)/efeitos adversos , Análise Fatorial , Feminino , Humanos , Estimativa de Kaplan-Meier , Acetato de Medroxiprogesterona/efeitos adversos , México , Pessoa de Meia-Idade , Seleção de Pacientes , Análise de Componente Principal , Progestinas/efeitos adversos , Medição de Risco , Inquéritos e Questionários , Fatores de Tempo , Resultado do Tratamento , Sistema Vasomotor/fisiopatologia

RESUMO

OBJECTIVE: To assess the saliva levels of CXCL13, CXCL10, CCL2, CCL3, CXCL12 and CCL5 in patients with primary SS (pSS), patients with associated SS (aSS), patients with systemic autoimmune disease (SAD) without SS, pre-clinical SS and healthy controls. METHODS: We included 44 patients with pSS (Group A), 30 with aSS (Group B), 49 with SAD without SS (Group C), 14 patients with SAD and focal lip infiltrates, but who do not fulfil SS criteria (Group D, pre-clinical SS) and 32 healthy controls (Group E). Saliva samples were collected and analysed for chemokine levels by luminometry. We used descriptive statistics and the Mann-Whitney U-test and Kruskall-Wallis test. RESULTS: All the studied chemokines were found at low concentration in controls with the exception of CCL2. Patients with pSS had higher levels CXCL10 and CCL2 than controls (P = 0.05). However, they had similar levels of CXCL13, CCL5, CXCL12, CCL2 and CXCL10 than patients with aSS and SAD without SS. Patients with pre-clinical SS had higher levels of CXCL10 than patients with pSS (P = 0.03), aSS (P = 0.04) and controls (P = 0.001). CCL2 levels were higher in all patients with an autoimmune background when compared with controls (P < 0.05 for each comparison). CONCLUSION: We found no difference in salivary chemokines between patients neither with pSS or aSS nor in patients with SAD. CCL2 and CXCL10 were increased in all patients with an autoimmune background. CXCL10 was notably increased in pre-clinical SS, suggesting it could be an early inflammatory salivary biomarker.

Assuntos

Doenças Autoimunes/imunologia , Quimiocina CXCL12/metabolismo , Quimiocina CXCL13/metabolismo , Saliva/química , Síndrome de Sjogren/imunologia , Doenças Autoimunes/diagnóstico , Biomarcadores/análise , Biomarcadores/metabolismo , Estudos de Casos e Controles , Quimiocina CXCL12/análise , Quimiocina CXCL13/análise , Feminino , Humanos , Masculino , Valores de Referência , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Síndrome de Sjogren/diagnóstico , Estatísticas não Paramétricas

RESUMO

OBJECTIVE: To compare the socio-economic characteristics, clinical features and health-related quality of life in Hispanic SLE patients residing in Mexico and in the Southwest USA (Mexican and Texan, herein). METHODS: Mexican and Texan SLE patients (fulfilling ACR criteria) participating in separate longitudinal outcome studies were evaluated. Texan patients were randomly chosen to match total disease duration with the Mexican patients. Cross-sectional data for the Mexican patients were obtained by a US-trained investigator who had previously participated in data collection for the cohort to which the Texan patients belonged. Socio-economic and -demographic characteristics, clinical characteristics, disease activity (with SLAM-Revised), damage accrual (with SLICC/ACR Damage Index) and self-reported function (with Short Form-36) were compared between the two groups. RESULTS: Seventy Mexican patients were matched with either one or two Texan patients (n = 94) for a total of 164 patients. Mexican patients were younger. In age-adjusted analyses, the Mexican patients were more educated, had better health-related quality of life and overall less systemic SLE manifestations. Mexican patients were exposed more frequently to AZA. CONCLUSIONS: Texan patients had more severe disease than the Mexican patients. In multivariable analyses, Texan Hispanic ethnicity was significantly associated with high disease activity, but significance was not reached for damage. The discrepant findings observed between these two Hispanic groups of SLE patients may reflect socio-economic or biological factors. Given the global phenomenon of immigration, rheumatologists should be aware of the overall course and outcome of immigrant SLE patients if undesirable outcomes are to be prevented.

Assuntos

Emigração e Imigração , Hispânico ou Latino/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/etnologia , Adolescente , Adulto , Estudos Transversais , Uso de Medicamentos/estatística & dados numéricos , Escolaridade , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , México/etnologia , Qualidade de Vida , Características de Residência , Índice de Gravidade de Doença , Fatores Socioeconômicos , Texas , Adulto Jovem

Assuntos

Anticoncepção/métodos , Lúpus Eritematoso Sistêmico , Adulto , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Síndrome Antifosfolipídica/complicações , Método de Barreira Anticoncepção , Anticoncepcionais Orais Combinados/efeitos adversos , Anticoncepcionais Orais Combinados/farmacologia , Anticoncepcionais Orais Hormonais/efeitos adversos , Anticoncepcionais Orais Hormonais/farmacologia , Contraindicações , Feminino , Hormônios Esteroides Gonadais/efeitos adversos , Hormônios Esteroides Gonadais/metabolismo , Humanos , Dispositivos Intrauterinos de Cobre , Dispositivos Intrauterinos Medicados/efeitos adversos , Levanogestrel/efeitos adversos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/metabolismo , Guias de Prática Clínica como Assunto , Gravidez , Gravidez de Alto Risco , Progestinas/administração & dosagem , Progestinas/efeitos adversos , Progestinas/farmacologia , Trombofilia/induzido quimicamente , Trombofilia/etiologia

RESUMO

BACKGROUND: Patients with Systemic Lupus Erythematosus (SLE) that present with acute abdominal pain (AAP) represent a challenge for the general surgeon. The purpose of this study was to identify the major causes of AAP among these patients and to define the role of disease activity scores and the APACHE II score in identifying patients with an increased perioperative risk. METHODS: We conducted a prospective study of patients admitted to the ER with AAP and SLE in an 11-year period. Demographic, diagnostic, and treatment data were recorded. Systemic lupus erythematosus disease activity index (SLEDAI), systemic lupus international collaboration clinics damage index (SLICC/DI), and APACHE II Score were analyzed. The main outcome variables were morbidity and mortality within 30 days of admission. RESULTS: Seventy-three patients were included. Ninety-three percent were female. Most common causes of AAP were: pancreatitis (29%), intestinal ischemia (16%), gallbladder disease (15%), and appendicitis (14%). Most causes of AAP in patients with LES were not related to the disease. APACHE II score > 12 was statistically associated with the diagnosis of intestinal ischemia compared to other causes. No relationship was observed between SLEDAI and outcome. Furthermore, this index did not have impact on diagnosis or decision making. Overall morbidity was 57% and overall mortality 11%. On multivariate analysis, only APACHE II > 12 was associated with mortality (P = 0.0001). CONCLUSION: This is one of the largest series of AAP and SLE. Most common causes of AAP were pancreatitis and intestinal ischemia. APACHE II score in patients with intestinal ischemia was higher than those with serositis; further studies are needed to examine whether this score may help to differentiate these etiologies when CT findings are inconclusive. APACHE II score was the most important factor associated with mortality. Furthermore, a prompt diagnosis and an appropriate surgical management are essential in order to improve patient outcome.

Assuntos

Dor Abdominal/diagnóstico , Dor Abdominal/epidemiologia , Causas de Morte , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , APACHE , Dor Abdominal/terapia , Doença Aguda , Adolescente , Adulto , Distribuição por Idade , Idoso , Análise de Variância , Estudos de Coortes , Comorbidade , Diagnóstico Precoce , Serviço Hospitalar de Emergência , Feminino , Humanos , Incidência , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Probabilidade , Estudos Prospectivos , Índice de Gravidade de Doença , Distribuição por Sexo , Análise de Sobrevida , Adulto Jovem

Assuntos

Vasculite Associada ao Lúpus do Sistema Nervoso Central/classificação , Vasculite Associada ao Lúpus do Sistema Nervoso Central/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Autoanticorpos/fisiologia , Humanos , Vasculite Associada ao Lúpus do Sistema Nervoso Central/tratamento farmacológico

RESUMO

OBJECTIVE: To evaluate the effects of menopause hormonal therapy on disease activity in women with systemic lupus erythematosus (SLE). METHODS: We conducted a double-blind, randomized clinical trial involving 106 women with SLE who were in the menopausal transition or in early or late postmenopause. Patients received a continuous-sequential estrogen-progestogen regimen (n = 52) or placebo (n = 54). Disease activity was assessed at baseline and at 1, 2, 3, 6, 9, 12, 15, 18, 21, and 24 months, according to the SLE Disease Activity Index (SLEDAI). The primary outcome measure was global disease activity, estimated by measuring the area under the SLEDAI curve. Secondary outcome measures included maximum SLEDAI score, change in SLEDAI score, incidence of lupus flares, median time to flare, medication use, and adverse events. Results were studied using intent-to-treat analysis. RESULTS: At baseline, demographic and disease characteristics were similar in both groups. Mean +/- SD SLEDAI scores were 3.5 +/- 3.3 and 3.1 +/- 3.4 in the menopause hormonal therapy and placebo groups, respectively (P = 0.57). Disease activity remained mild and stable in both groups throughout the trial. There were no significant differences between the groups in global or maximum disease activity, incidence or probability of flares, or medication use. Median time to flare was 3 months in both groups. Thromboses occurred in 3 patients who received menopause hormonal therapy and in 1 patient who received placebo. One patient in each group died during the trial due to sepsis. CONCLUSION: Menopause hormonal therapy did not alter disease activity during 2 years of treatment. However, an apparently increased risk of thrombosis seems to be a real threat in women with SLE who receive menopausal hormone therapy.

Assuntos

Terapia de Reposição de Estrogênios , Lúpus Eritematoso Sistêmico/fisiopatologia , Menopausa , Progestinas/uso terapêutico , Adulto , Idoso , Método Duplo-Cego , Seguimentos , Terapia de Reposição Hormonal , Humanos , Pessoa de Meia-Idade

RESUMO

Primary immunodeficiencies as well as autoimmune diseases have been associated to X chromosome abnormalities. Furthermore, the functional biology of the X chromosome is unique because genes located in this chromosome can undergo inactivation, and subsequently transcriptional silencing. Non-random X chromosome inactivation has been hypothesized to be involved in the development of autoimmunity. Recently X chromosome monosomy has also been proposed as a common etiologic mechanism for some autoimmune diseases. Herein, we review some of these findings above mentioned.

Assuntos

Autoimunidade , Cromossomos Humanos X , Aneuploidia , Animais , Epigênese Genética , Feminino , Humanos , Masculino , Inativação do Cromossomo X

RESUMO

Chemokines and cytokines play an important role in the inflammatory development and progression of autoimmune diseases. The aim of the present study was to evaluate the role of MCP-1, SDF-1, and RANTES polymorphisms as susceptibility markers for systemic lupus erythematosus (SLE) in a group of Mexican patients. MCP-1-2518, SDF-1 G801A, and RANTES-28 polymorphisms were determined in 242 patients with SLE and 220 ethnically matched healthy controls by the polymerase chain reaction-restriction fragment length polymorphism technique. The differences between patients and healthy controls were evaluated by chi(2), Fisher's exact test, and Woolf method for odds ratio. A moderately increased frequency of MCP-1-2518 A allele (p = 0.033, pC = NS) and AA genotype (p = 0.017, pC = NS) existed in SLE patients compared with healthy controls. There was a relationship between polymorphisms and some clinical and laboratory characteristics. SLE patients with and without antiphospholipid syndrome demonstrated different distribution of SDF-1 G801A genotype frequencies. On the other hand, patients with leukopenia, anti-dsDNA, and antiphospholipid autoantibodies demonstrated different MCP-1-2518 genotype distribution compared with patients without these features. Our results suggest that MCP-1 polymorphism is moderately associated with the genetic susceptibility to SLE in Mexican individuals. The polymorphisms could be related to specific clinical and laboratory characteristics in these patients.

Assuntos

Quimiocina CCL2/genética , Quimiocina CCL5/genética , Quimiocina CXCL12/genética , Lúpus Eritematoso Sistêmico/genética , Polimorfismo Genético , Adulto , Alelos , Feminino , Frequência do Gene , Predisposição Genética para Doença , Genótipo , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade

RESUMO

OBJECTIVE: To establish the association between oral pathology and pneumonia in patients with systemic lupus erythematosus (SLE). METHODS: Thirty women with SLE, consecutively admitted for hospitalization because of pneumonia, and 60 noninfected controls with SLE (30 hospitalized and 30 ambulatory), matched by age, sex, and date of hospitalization to the cases, were enrolled. At entry, information about sociodemographic variables, traditional infection risk factors, SLE characteristics, treatment, and comorbidity was gathered by medical chart review. In every patient, one rheumatologist performed a complete physical examination, and assessed disease activity and chronic damage using validated indices; and one periodontist performed a standardized oral health evaluation including the use of 6 international oral health indices. RESULTS: Twenty-eight patients with community-acquired and 2 patients with nosocomial pneumonias were included. Age of the total study population was 38.8 +/- 14.6 years, mean number of SLE criteria 6.3 +/-1.95, and disease duration 6.6 +/- 7.2 years, with no differences among the 3 groups. Cases had greater disease activity and damage, and were taking higher doses of prednisone than ambulatory controls (p

Assuntos

Cárie Dentária/etiologia , Lúpus Eritematoso Sistêmico/complicações , Pneumonia/etiologia , Adulto , Anti-Inflamatórios/uso terapêutico , Estudos de Casos e Controles , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Pessoa de Meia-Idade , Análise Multivariada , Higiene Bucal/efeitos adversos , Prednisona/uso terapêutico , Fatores de Risco

RESUMO

BACKGROUND: The effects of estrogen-containing contraceptives on disease activity in women with systemic lupus erythematosus have not been determined. METHODS: We conducted a single-blind clinical trial involving 162 women with systemic lupus erythematosus who were randomly assigned to combined oral contraceptives, a progestin-only pill, or a copper intrauterine device (IUD). Disease activity was assessed at 0, 1, 2, 3, 6, 9, and 12 months according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). The primary outcome was global disease activity, which we estimated by measuring the area under the SLEDAI curve. Secondary outcomes included the maximum SLEDAI score, change in SLEDAI score, incidence of lupus flares, median time to first flare, systemic lupus erythematosus treatment, and adverse events. The results were analyzed by the intention-to-treat method. RESULTS: At baseline, all demographic features and disease characteristics were similar in the three groups. The mean (+/-SD) SLEDAI score was 6.1+/-5.6 in the group assigned to combined oral contraceptives, 6.4+/-4.6 in the group assigned to the progestin-only pill, and 5.0+/-5.3 in the group assigned to the IUD (54 patients in each group) (P=0.36). Disease activity remained mild and stable in all groups throughout the trial. There were no significant differences among the groups during the trial in global or maximum disease activity, incidence or probability of flares, or medication use. The median time to the first flare was three months in all groups. Thromboses occurred in four patients (two in each of the two groups receiving hormones), and severe infections were more frequent in the IUD group. One patient receiving combined oral contraceptives died from amoxicillin-related severe neutropenia. CONCLUSIONS: Global disease activity, maximum SLEDAI score, incidence of flares, time to first flare, and incidence of adverse events were similar among women with systemic lupus erythematosus, irrespective of the type of contraceptive they were using.

Assuntos

Anticoncepcionais Orais , Dispositivos Intrauterinos , Levanogestrel , Lúpus Eritematoso Sistêmico , Adulto , Anticoncepcionais Orais/efeitos adversos , Etinilestradiol/efeitos adversos , Feminino , Humanos , Levanogestrel/efeitos adversos , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/fisiopatologia , Gravidez , Índice de Gravidade de Doença , Método Simples-Cego