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3.
Lupus ; 28(11): 1344-1349, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31551028

RESUMO

OBJECTIVE: The aim of this study was to determine whether remission and low disease activity state protect systemic lupus erythematosus patients from being hospitalized. MATERIALS AND METHODS: Patients from the Almenara Lupus Cohort were included. Visits were performed every 6 months. Variables were measured at each visit. Hospitalizations were evaluated in the interval between two visits. Remission was defined as: a SLEDAI-2 K of 0, prednisone ≤5 mg/day and immunosuppressants on maintenance dose; low disease activity state as: a SLEDAI-2 K of ≤4, prednisone ≤7.5 mg/day and immunosuppressants on maintenance dose. Univariable and multivariable interval-censored survival regression models were used. In multivariable analysis, possible confounders were gender, age at diagnosis, socioeconomic status, educational level, disease duration, antimalarial use, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SDI) and Charlson comorbidity index. Confounders were determined in the same visit as disease activity state. RESULTS: Of the 308 patients, 92.5% of them (n = 285) were women, had a mean age at diagnosis of 34.8 (13.4) years and a disease duration of 7.7 (6.5) years. At baseline the mean SDI was 1.13 (1.34). A total of 163 of the patients were hospitalized. In the multivariable analysis remission (hazard ratio 0.445 (0.274-0.725), P = 0.001) and low disease activity state (relative risk 0.504 (0.336-0.757), P = 0.001) at baseline were found to decrease the risk of hospitalization in systemic lupus erythematosus patients. A total of 158 hospitalizations presented a discernible cause. Disease activity was the most common cause of hospitalization, with 84 admissions (53.16%), the majority, 38, was due to active kidney disease (45.23%). CONCLUSION: Remission and low disease activity state decreased the risk of hospitalizations in these systemic lupus erythematosus patients. Disease activity, particularly renal, was the most frequent cause of hospitalization.


Assuntos
Hospitalização/estatística & dados numéricos , Imunossupressores/administração & dosagem , Nefropatias/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Estudos de Coortes , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Indução de Remissão , Índice de Gravidade de Doença , Fatores Socioeconômicos , Adulto Jovem
4.
Isr Med Assoc J ; 21(7): 464-470, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31507122

RESUMO

BACKGROUND: Rheumatic diseases commonly affect women of childbearing age, when women may be contemplating pregnancy or they discover an unplanned pregnancy. Therefore, specific issues about pregnancy planning and management are commonly encountered in patients during these times. Knowledge of the effect of pregnancy on disease activity is important for counseling. This review summarizes recent data on the course of different rheumatic diseases during pregnancy and the postpartum period. Rheumatoid arthritis and systemic lupus erythematosus are the most commonly investigated diseases. Data are increasing about spondyloarthritis. Sparse data are available for other rheumatic diseases. Despite the differences in these diseases and the various courses these disease take during pregnancy, a common feature is that active maternal disease in the months prior to conception increases the risk of flares during pregnancy, which in turn can lead to adverse pregnancy outcomes. Therefore, maternal and fetal health can be optimized if conception is planned when disease is inactive so that a treatment regimen can be maintained throughout pregnancy.


Assuntos
Complicações na Gravidez/fisiopatologia , Resultado da Gravidez , Doenças Reumáticas/fisiopatologia , Artrite Reumatoide/fisiopatologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologia , Período Pós-Parto , Gravidez
6.
Croat Med J ; 60(4): 333-344, 2019 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-31483119

RESUMO

AIM: To validate Systemic Lupus International Collaborating Clinics (SLICC)-12 and American College of Rheumatology (ACR)-97 classification criteria on a patient cohort from the University Hospital Center Zagreb. METHODS: This retrospective study, conducted from 2014 to 2016, involved 308 patients with systemic lupus erythematosus (SLE) (n=146) and SLE-allied conditions (n=162). Patients' medical charts were evaluated by an expert rheumatologist to confirm the clinical diagnosis, regardless of the number of the ACR-97 criteria met. Overall sensitivity and specificity, as well as the sensitivity and specificity according to disease duration, were compared between ACR-97 and SLICC-12 classifications. Predictive value for SLE for both classifications was assessed using logistic regression and receiver operating characteristic (ROC) curves. RESULTS: The SLICC-12 criteria had significantly higher sensitivity in early disase, which increased with disease duration. The ACR-97 criteria had higher specificity. The specificity of the SLICC-12 criteria was low and decreased with disease duration. Regression analysis demonstrated the superiority of the SLICC-12 classification criteria over the ACR-97 criteria, with areas under the ROC curve of 0.801 and 0.780, respectively. CONCLUSION: Although the SLICC-12 criteria were superior to the ACR-97 and were more sensitive for diagnosing early SLE, their specificity in our population was too low. The sensitivity of the SLICC-12 classification is increased by better defined clinical features within each criterion. Our results contribute to the current initiative for developing new criteria for SLE.


Assuntos
Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Hospitais Universitários , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Curva ROC , Encaminhamento e Consulta , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Adulto Jovem
7.
Med Sci Monit ; 25: 5929-5933, 2019 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-31395849

RESUMO

BACKGROUND The objective of this study was to detect the level of olfactory function in patients with systemic lupus erythematosus (SLE) and to explore the relationship between impaired olfactory function and anti-ribosomal P protein antibody (ARPA), disease duration, and age. MATERIAL AND METHODS The level of olfactory function in 65 patients with SLE and 50 healthy participants was detected using the Connecticut Chemosensory Clinical Research Center (CCCRC) method; serum ARPA levels in SLE patients and the healthy control group were detected by enzyme-linked immunosorbent assay (ELISA). RESULTS CCCRC scores in the active SLE group was lower than that in the inactive SLE and healthy control groups (P<0.01). In SLE patients, the CCCRC scores of ARPA-positive patients were lower than those of ARPA-negative patients (P<0.01). A negative correlation was discovered between CCCRC scores and ARPA serum levels in SLE patients. Multiple linear regression analyses showed a correlation among the CCCRC score, age, and ARPA. CONCLUSIONS Olfactory dysfunction was found in patients with active SLE; which correlated with SLE disease activity and ARPA levels.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Percepção Olfatória/fisiologia , Olfato/fisiologia , Adolescente , Adulto , Fatores Etários , Idoso , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Proteínas Ribossômicas/imunologia , Índice de Gravidade de Doença
8.
Lupus ; 28(11): 1285-1293, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31399014

RESUMO

Autoantibodies (AA) and antinuclear antibodies (ANA) serve as key diagnostic and classification criteria for systemic lupus erythematosus (SLE). More than 200 different AA have been reported in SLE, although only a handful (<20) are considered "mainstream" because they are widely and routinely used in diagnostic, research and clinical medicine. Although the vast majority of AA have been relegated to the diminished status of "orphan" AA, some serve as predictors of SLE because they first appear in very early or subclinical SLE. Some AA are pathogenic, whereas others are thought to protect against or ameliorate disease progression and, hence, taken together can be used as predictive biomarkers of prognosis. Although studies have shown that specific AA are detected in the preclinical phase of SLE and are biomarkers of increased risk of developing the disease, AA are currently not widely used to predict very early SLE in individuals who have low pretest probability of disease. With the advent of multianalyte arrays with analytic algorithms, emerging evidence indicates that when certain combinations of biomarkers, such as the interferon signature and stem cell factor accompany AA and ANA, the predictive power for SLE is markedly increased.


Assuntos
Anticorpos Antinucleares/imunologia , Autoanticorpos/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Biomarcadores/metabolismo , Progressão da Doença , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/fisiopatologia , Prognóstico
9.
Lupus ; 28(11): 1360-1367, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31382851

RESUMO

OBJECTIVE: The objective of this article is to investigate the distinct features, morbidity and mortality of systemic lupus erythematosus (SLE) patients registered in rheumatology tertiary centers of Shiraz University of Medical Sciences from 2008 to 2018. METHOD: A cross-sectional study was conducted on registered patients by reviewing their medical records. The clinical presentations were classified based on each organ's distinct involvement. RESULT: Data of 1322 patients with a mean age of 40 at the time of the study were reviewed. The most common primary manifestation of the disease was arthralgia (37.5%), and the most common clinical presentation in course of the disease was skin involvement (61.8%). The most common cause of hospital admission was disease flare (27.8% (108/388)), and mortality was mainly due to pulmonary complications (27.5% (8/29)). Patients who had hypothyroidism had statistically significantly lower rates of constitutional symptoms (p = 0.006), gastrointestinal involvement (p = 0.024), hypertension (p = 0.002), headache (p = 0.019), depression (p = 0.049) and anemia (p = 0.014). Patients who presented with malar rash and photosensitivity in their course of disease were detected to have statistically significantly lower ages of disease onset (p = 0.013 and 0.003, respectively). CONCLUSION: This study is an update to an epidemiologic study conducted in 2008 in the south of Iran. By comparing results between the two studies, we have tried to investigate the change in morbidity and mortality of SLE during these years.


Assuntos
Artralgia/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Idade de Início , Artralgia/etiologia , Estudos Transversais , Feminino , Humanos , Irã (Geográfico) , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
10.
Lupus ; 28(10): 1250-1254, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31382852

RESUMO

OBJECTIVE: The objective of this study was to determine the safety of tattoos in patients with systemic lupus erythematosus (SLE). METHODS: Patients (N = 147; ≤55 years; 92% women) were asked if they had tattoos. The characteristics of the tattoos and the immediate complications were investigated and compared with those of a matched control group. We examined retrospectively after the tattoo was completed whether there had been flare-ups or increased organ damage (Systemic Lupus International Collaborating Clinics/American Collage of Rheumatology Damage Index (SDI)). Finally, we compared the SLE-related characteristics of patients with and without tattoos. RESULTS: Twenty-eight patients (19%, 26 women, median (interquartile range) age 33 (25-42) years, 65 tattoos in total) had ≥1 tattoo. At the time the tattoo was done the median (interquartile range) SLEDAI and SDI were 2 (0-2) and 0 (0-1), respectively. The characteristics of the tattoos were similar to those of controls. No patients experienced acute complications. After a median follow-up of 17 (12-20) months (3 (2-4) visits/year) four patients had five mild-to-moderate flare-ups. The median time between the tattoo and the flare-up was 9 (6-14) months. No increase in SDI was observed. The SLE-related characteristics of patients with and without tattoos were similar. CONCLUSION: Tattoos seem to be safe in SLE patients with inactive or low active disease.


Assuntos
Lúpus Eritematoso Sistêmico/fisiopatologia , Tatuagem/estatística & dados numéricos , Adulto , Estudos de Casos e Controles , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Tatuagem/efeitos adversos
11.
Lupus ; 28(10): 1224-1232, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31409184

RESUMO

OBJECTIVE: Juvenile-onset systemic lupus erythematosus (JoSLE) is associated with low bone mass for age and fractures; nevertheless, risk factors for bone impairment are poorly understood. The aim of this study was to evaluate risk factors for bone mass loss in JoSLE patients. METHODS: Forty-nine female JoSLE patients were evaluated at baseline and after a 3.5-year follow-up regarding clinical, laboratory (including bone turnover markers), areal bone mineral density (aBMD) and bone microarchitecture parameters using high-resolution peripheral quantitative computed tomography (HR-pQCT). Based on the difference between final and baseline aBMD value, the patients were divided into three groups: aBMD gain (BG), aBMD loss (BL) and aBMD no change (NC). RESULTS: The mean patient age was 18.7 ± 3.3 years. Sixty-one percent of patients presented with aBMD gain, 18.4% aBMD loss, and 20.4% remained stable during this follow-up period. Comparing the BL with the BG group, there was a higher frequency of alcohol consumption (p = 0.009), a higher frequency of inadequate calcium intake (p = 0.047) and lower levels of baseline procollagen type 1 amino-terminal propeptide (P1NP) (p = 0.036) in the BL group. Moreover, worsening of HR-pQCT parameters trabecular volumetric density (p = 0.003) and cortical thickness (p = 0.009) was observed in the BL group. In addition, a higher frequency of renal activity was observed comparing the BL + NC with the BG group (p = 0.036). CONCLUSIONS: This is the first longitudinal study that has analyzed the risk factors of bone loss in JoSLE patients. The authors emphasize the importance of evaluating lifestyle habits and renal disease activity in these young women. Furthermore, this study suggests that trabecular and cortical compartments deteriorated, and low levels of P1NP may be a predictor of bone impairment in JoSLE.


Assuntos
Densidade Óssea/fisiologia , Osso e Ossos/patologia , Lúpus Eritematoso Sistêmico/complicações , Adolescente , Consumo de Bebidas Alcoólicas/epidemiologia , Osso e Ossos/metabolismo , Cálcio/administração & dosagem , Feminino , Seguimentos , Humanos , Nefropatias/etiologia , Nefropatias/fisiopatologia , Estilo de Vida , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/fisiopatologia , Estudos Prospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X , Adulto Jovem
13.
Lupus ; 28(10): 1233-1242, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31403902

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) patients have lower bone mineral density (BMD) compared with healthy individuals because of general, genetic, disease and medication-related factors. The disturbance of the receptor activator of nuclear factor-κB ligand (RANKL)/osteoprotegerin (OPG) ratio has been reported to be associated with low BMD in many disorders in adults and children alike. OBJECTIVES: The objectives of this study were (i) to assess serum OPG, RANKL and RANKL/OPG ratio levels in SLE children and controls, (ii) to determine whether the cumulative glucocorticoid (CGCS) dose had any effect on the concentration of serum RANKL, OPG and RANKL/OPG ratio, and (iii) to determine the relation of these parameters to BMD. METHODS: We evaluated 50 SLE children and 50 age- and sex-matched healthy controls. RANKL and OPG were assessed in serum and compared between patients and controls. For SLE patients, a univariate followed by multivariable analysis were carried out to detect the possible predictors of the changes in RANKL, OPG and RANKL/OPG ratio levels. Lumbar BMD for all patients was assessed by dual-energy X-ray absorptiometry (DXA) scan and then correlated to different probable correlated factors. RESULTS: RANKL, OPG and RANKL/OPG ratio were significantly higher in SLE patients (p ≤ 0.001). Univariate analysis showed significant correlations of RANKL with CGCS (p ≤ 0.001) and with DXA scan z-score (p = 0.007): OPG was significantly correlated to Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (p = 0.001) and anti-double-stranded DNA (p = 0.001), whereas RANKL/OPG was significantly correlated to duration of illness and DXA z-score (p = 0.002). The multivariable analysis showed that DXA z-score was an independent predictor of RANKL and RANKL/OPG ratio (p = 0.019 and 0.008, respectively), whereas SLEDAI score was an independent predictor of OPG levels. BMD was negatively correlated to disease duration (p = 0.008) and CGCS dose (p = 0.015), but no significant correlation has been found between BMD and cumulative SLEDAI score (p = 0.29). CONCLUSIONS: Serum RANKL/OPG ratio is elevated in Egyptian children with SLE and is considered a risk factor for reduced bone mass in these children. Other risk factors for low BMD include high CGCS dose and disease duration, supporting that osteoporosis in SLE is multifactorial.


Assuntos
Densidade Óssea/fisiologia , Lúpus Eritematoso Sistêmico/sangue , Osteoprotegerina/sangue , Ligante RANK/sangue , Absorciometria de Fóton , Adolescente , Estudos de Casos e Controles , Criança , Relação Dose-Resposta a Droga , Egito , Feminino , Glucocorticoides/administração & dosagem , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Osteoporose/etiologia , Fatores de Risco , Índice de Gravidade de Doença
14.
BMC Gastroenterol ; 19(1): 154, 2019 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-31455284

RESUMO

BACKGROUND: Systemic lupus erythematosus is an autoimmune disease which can affect multiple organs, resulting in significant mortality and morbidity. Lupus enteritis is one of the rare complications of SLE, defined as vasculitis of the intestinal tract, with supportive biopsy findings and/or image. However, lupus enteritis is seldom confirmed on histology or image and the changes of intestinal mucosa are nonspecific. Crohn's disease is a chronic inflammatory disorder of the gastrointestinal tract which affects any part of the gastrointestinal tract. The diagnosis of CD is confirmed by clinical evaluation and a combination of endoscopic, histology, radiology, and/or biochemical investigations. CASE PRESENTATION: Here we report a rare case of a 71-years-old Chinese male has been diagnosed with lupus enteritis which similar to CD in the aspects of endoscopic, histology, and radiology. So far, there are no relevant cases reported. CONCLUSIONS: The endoscopic appearance of lupus enteritis is nonspecific, on the basis of our case, the features of lupus enteritis can be described as spacious, clean and no moss ulcers which discontinuous involved all gastrointestinal tract.


Assuntos
Doença de Crohn/diagnóstico , Endoscopia do Sistema Digestório/métodos , Enterite , Trato Gastrointestinal , Lúpus Eritematoso Sistêmico , Idoso , Anticorpos Antinucleares/sangue , Anticorpos Antifosfolipídeos/sangue , Diagnóstico Diferencial , Enterite/diagnóstico , Enterite/etiologia , Enterite/fisiopatologia , Trato Gastrointestinal/diagnóstico por imagem , Trato Gastrointestinal/patologia , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/terapia , Masculino , Tomografia Computadorizada por Raios X/métodos
15.
Lupus ; 28(9): 1148-1153, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31369342

RESUMO

OBJECTIVE: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with treatment manifestations that can cause changes in appearance, including skin rashes, alopecia, vitiligo, and scars. SLE has been shown to adversely impact body image outcomes, and previous research has identified that greater disease activity is associated with worse body image outcomes which, in turn, are associated with greater depressive symptoms. For patients with SLE who also experience significant pain, poor body image outcomes may further compromise wellbeing and lead to greater depressive symptoms. The role of pain in body image has not been explored in SLE. Thus, the present study examined whether body image (specifically, body image-related quality of life) serves as a mediator of the relationship between pain and depressive symptoms among patients with SLE. METHODS: Multiple mediation analysis was used to examine the hypothesis that body image-related quality of life mediates the relationship between pain and depressive symptoms in a sample of patients with SLE (N = 135) from an urban region in Los Angeles, California. RESULTS: The sample was predominately female (92.6%) with a mean disease duration of approximately 17 years. Approximately one-quarter of the sample had elevated depressive symptoms. Body image-related quality of life was a significant mediator in the relationship between pain and depressive symptoms. The model accounted for 51% of the total variance in depressive symptoms (R2 = 0.51). CONCLUSION: This cross-sectional study suggested that body image-related quality of life may mediate the effects of pain on depressive symptoms among patients with SLE.


Assuntos
Imagem Corporal/psicologia , Depressão/epidemiologia , Lúpus Eritematoso Sistêmico/psicologia , Dor/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Depressão/etiologia , Feminino , Humanos , Los Angeles , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Qualidade de Vida , Adulto Jovem
16.
Lupus ; 28(9): 1158-1166, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299879

RESUMO

INTRODUCTION: Although extensively characterized in the outpatient setting, systemic lupus erythematosus (SLE) in the hospitalization wards is still scarcely portrayed, particularly in the perspective of its evolution over the years. METHODS: Retrospective analysis of SLE patients hospitalized in the Department of Autoimmune Diseases of a university hospital during a 20-year period (1995-2015), describing hospitalization characteristics, causes and predictors of outcome. RESULTS: A total of 814 hospitalizations concerning 339 patients were analysed. The main causes of admission were flare (40.2%), infection (19.2%), diagnostic procedures (18.8%) and thrombotic events (5.4%). Therapy with cyclophosphamide (odds ratio (OR) 1.908, p = 0.047) was associated with admission due to infection, while antimalarials displayed a protective effect (OR 0.649, p = 0.024). Nearly 3.9% of patients required admission to an intensive care unit, with associated antiphospholipid syndrome (OR 7.385, p = 0.04) standing as a predicting factor for this outcome. Readmission at 30 days occurred in 5.8% of patients, with thrombocytopenia (OR 6.007, p = 0.002) and renal involvement (OR 3.362, p = 0.032) featuring as predicting factors. Eight patients died, with antiphospholipid syndrome (OR 26.814, p = 0.02) and thrombocytopenia (OR 31.523, p = 0.01) being associated with mortality. There was no significant variation in patients' demographics or admission causes across the 20-year period, except for a decrease in admissions due to thrombotic and musculoskeletal causes. Recently, an increase in the use of mycophenolate mofetil and lower doses of glucocorticoids were noted. CONCLUSION: While demographics of SLE hospitalizations have not markedly changed over the past 20 years, changes in therapy patterns were observed. Thrombocytopenia, antiphospholipid syndrome and renal involvement featured as predictors of poor outcome.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/terapia , Trombocitopenia/epidemiologia , Adulto , Síndrome Antifosfolipídica/etiologia , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Espanha , Trombocitopenia/etiologia
17.
Lupus ; 28(9): 1120-1127, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299881

RESUMO

Sensing self-nucleic acids through toll-like receptors in plasmacytoid dendritic cells (pDCs), and the dysregulated type I IFN production, represent pathogenic events in the development of the autoimmune responses in systemic lupus erythematosus (SLE). Production of high-mobility group box-1 protein (HMGB1) promotes type I IFN response in pDCs. To better understand the active pathogenic mechanism of SLE, we measured serum levels of HMGB1, thrombomodulin, and cytokines (IL-1ß, IL-2, IL-4, IL-5, IL-6, IL-10, IL-13, IL-17A, IL-17F, IFNα, IFNγ, TNFα) in 35 patients with SLE. Serum HMGB1 and IFNα were significantly higher in patients with active SLE (SLE Disease Activity Index (SLEDAI) score ≥ 6) compared with healthy donors or patients with inactive SLE. Furthermore, the HMGB1 levels were significantly correlated with IFNα levels. By qualitative analysis, the detection of serum IFNα or HMGB1 suggests active SLE and the presence of SLE-related arthritis, fever, and urinary abnormality out of SLEDAI manifestations. Collectively, HMGB1 and IFNα levels are biomarkers reflecting disease activity, and qualitative analysis of IFNα or HMGB1 is a useful screening test to estimate SLE severity and manifestations. Our results suggest the clinical significance of type I IFNs and HMGB1 as key molecules promoting the autoimmune process in SLE.


Assuntos
Citocinas/sangue , Proteína HMGB1/sangue , Lúpus Eritematoso Sistêmico/fisiopatologia , Trombomodulina/sangue , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Humanos , Interferon-alfa/sangue , Lúpus Eritematoso Sistêmico/sangue , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença
18.
Lupus ; 28(9): 1167-1173, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299882

RESUMO

Management of systemic lupus erythematosus patients is challenging because of disease heterogeneity. Although treatment of renal nephritis is more standardized, treating non-renal lupus activity remains controversial. Our objective was to identify non-renal, non-neurologic persistent active systemic lupus erythematosus patients in our cohort and described therapeutic behaviors in them. All systemic lupus erythematosus patients (American College of Rheumatology and/or Systemic Lupus Erythematosus International Collaborating Clinics criteria) seen at a university hospital between 2000 and 2017 were included and electronic medical records manually reviewed. Persistent lupus activity was defined as a patient with a Systemic Lupus Erythematosus Disease Activity Index score ≥ 6 (without renal and central nervous system manifestations) despite being on a stable treatment regimen for ≥ 30 days. Stable treatment could include prednisone alone (7.5-40 mg/d) or combined with antimalarial drugs and immunosuppressant therapies. A total of 257 lupus patients were included, 230 females (89.5%, 95% confidence interval 85.1-92.7), mean age at diagnosis 29.9 years (SD 16.4). After a median cohort follow-up of 5.7 years (interquartile range 2.4-10.2), 14 patients (5.4%, 95% confidence interval 3.2-9.0) showed persistent non-renal non neurologic lupus activity, with a median disease duration of 11.3 years (interquartile range 3.6-19.4). At that time, 12/14 (85.7 %, 95% confidence interval 52.6-97.0%) had low complement and 11/14 (78.6 %, 95% confidence interval 46.5-93.9%) had positive antiDNA antibodies. The main reasons for being refractory were mucocutaneous disease (50%, 95% confidence interval 23.5-76.5) and arthritis (42.9%, 95% confidence interval 18.5-71.2). Therapeutic choices after being refractory were: only increasing corticosteroid dose in one patient, starting rituximab in four, belimumab in eight, and in one mycophenolate and rituximab; with good response in all of them. In conclusion, 5.4% of systemic lupus erythematosus patients in our cohort were considered to have non-renal non neurologic persistent lupus activity, with mucocutaneous and arthritis the main manifestations. In total, 92.8% of these patients started a biologic treatment at this point (rituximab or belimumab).


Assuntos
Glucocorticoides/administração & dosagem , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adolescente , Adulto , Anticorpos Monoclonais Humanizados/administração & dosagem , Antimaláricos/administração & dosagem , Argentina , Estudos de Coortes , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Feminino , Seguimentos , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Prednisona/administração & dosagem , Estudos Retrospectivos , Rituximab/administração & dosagem , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto Jovem
19.
Lupus ; 28(9): 1141-1147, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31256746

RESUMO

OBJECTIVE: To identify determinants and outcomes associated with infection in paediatric systemic lupus erythematosus (SLE) patients at admission and during hospitalization in intensive care units (ICUs). PATIENTS AND METHODS: A retrospective cohort study of paediatric SLE patients admitted to two ICUs was conducted. Frequency and risk factors of infection as well as mortality were studied. RESULTS: Seventy-three infection episodes amongst 55 patients were analysed. The median age was 14.4 years (IQR 12.5-16). The median SLEDAI was 16 (IQR 12-20). Twenty-nine episodes were documented at admission; the CRP was higher in these patients (6.58 versus 1.04 mg/dl, p<0.001) than in non-infected patients, even after multivariate adjustment (OR 8.6, 95% CI = 2.1-34.8, p = 0.003). Twenty-five (34.7%) episodes occurred during hospitalization. Lupus activity (OR 1.14, 95% CI = 1.01-1.27, p = 0.029), cyclophosphamide (OR 17.9, 95% CI = 2-156, p = 0.009) and mechanical ventilation (OR 16, 95% CI = 2.1-122, p = 0.008) were associated with infection. Ten episodes (14%) led to death. Admission to the ICU due to infection was strongly associated with mortality (90% versus 31.8%, OR 19.4, 95% CI = 2.3-163, p = 0.006). CONCLUSION: In paediatric lupus patients admitted to the ICU, elevated CRP should alert clinicians to possible infection. During hospitalization, SLE activity and cyclophosphamide were associated with infection. Infection at admission to the ICU was strongly associated with mortality.


Assuntos
Hospitalização/estatística & dados numéricos , Unidades de Terapia Intensiva/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/complicações , Adolescente , Estudos de Coortes , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
20.
Lupus ; 28(10): 1261-1272, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31296137

RESUMO

BACKGROUND: Although the survival of patients with systemic lupus erythematosus (SLE) has improved, irreversible organ damage remains a critical concern. We aimed to characterize damage accrual and its clinical associations and causes of death in Swedish patients. METHODS: Accumulation of damage was evaluated in 543 consecutively recruited and well-characterized cases during 1998-2017. The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI) was used to estimate damage. RESULTS: Organ damage (SDI ≥ 1) was observed in 59%, and extensive damage (SDI ≥ 3) in 25% of cases. SDI ≥ 1 was significantly associated with higher age at onset, SLE duration, the number of fulfilled SLICC criteria, neurologic disorder, antiphospholipid antibody syndrome (APS), hypertension, hyperlipidemia, depression and secondary Sjögren's syndrome (SS). In addition, SDI ≥ 3 was associated with serositis, renal and haematological disorders and interstitial lung disease. A multiple regression model identified not only well-known risk factors like APS, antihypertensives and corticosteroids, but pericarditis, haemolytic anaemia, lymphopenia and myositis as being linked to SDI. Malignancy, infection and cardiovascular disease were the leading causes of death. CONCLUSIONS: After a mean SLE duration of 17 years, the majority of today's Swedish SLE patients have accrued damage. We confirm previous observations and report some novel findings regarding disease phenotypes and damage accrual.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Suécia , Adulto Jovem
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