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OBJECTIVE: The aim of this study was to assess right atrial (RA) function, including RA phase strain, via speckle-tracking echocardiography (STE) in a cohort of systemic lupus erythematosus (SLE) patients with pulmonary arterial hypertension (PAH) and in particular to explore the relationship between RA phase strain and the occurrence of cardiovascular events. METHODS: STE analyses of RA function were evaluated in patients with SLE-PAH and in 33 healthy control subjects. Clinical associations, serum biomarkers, echocardiographic data, survival times, and adverse cardiovascular events were evaluated. RESULTS: A total of 66 patients with SLE-PAH were enrolled; they were divided into two groups based on the occurrence of adverse clinical events. RA phase strain was significantly reduced in patients with events than in patients without events. The endpoint was defined as the combined outcome of all-cause mortality, right heart failure, and rehospitalization due to disease progression. During a mean follow-up of 17.2 ± 9.9 months, 23 patients (35%) reached the endpoint. Compared with patients with RA reservoir strain (RASr) ≥33.45%, patients with RASr < 33.45% had more adverse long-term outcomes (log rank p < .0001). RASr was independently associated with adverse clinical outcomes according to multivariate analysis (p = .010). CONCLUSION: Our data suggest that RA function has prognostic value for SLE-PAH patients, and strain analysis revealed that the worse the RA function is, the worse the prognosis.
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Ecocardiografía , Atrios Cardíacos , Lupus Eritematoso Sistémico , Humanos , Femenino , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/fisiopatología , Pronóstico , Ecocardiografía/métodos , Persona de Mediana Edad , Adulto , Hipertensión Arterial Pulmonar/fisiopatología , Hipertensión Arterial Pulmonar/complicaciones , Hipertensión Arterial Pulmonar/etiología , Hipertensión Arterial Pulmonar/sangre , Función del Atrio Derecho/fisiología , Hipertensión Pulmonar/fisiopatología , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/etiología , Estudios de SeguimientoRESUMEN
PURPOSE: This study investigated the topological structural characteristics of systemic lupus erythematosus (SLE) with and without neuropsychiatric symptoms (NPSLE and non-NPSLE), and explore their clinical implications. METHODS: We prospectively recruited 50 patients with SLE (21 non-NPSLE and 29 NPSLE) and 32 age-matched healthy controls (HCs), using MRI diffusion tensor imaging. Individual structural networks were constructed using fibre numbers between brain areas as edge weights. Global metrics (eg, small-worldness, global efficiency) and local network properties (eg, degree centrality, nodal efficiency) were computed. Group comparisons of network characteristics were conducted. Clinical correlations were assessed using partial correlation, and differentiation between non-NPSLE and NPSLE was performed using support vector classification. RESULTS: Patients with oth non-NPSLE and NPSLE exhibited significant global and local topological alterations compared with HCs. These changes were more pronounced in NPSLE, particularly affecting the default mode and sensorimotor networks. Topological changes in patients with SLE correlated with lesion burdens and clinical parameters such as disease duration and the systemic lupus international collaborating clinics damage index. The identified topological features enabled accurate differentiation between non-NPSLE and NPSLE with 87% accuracy. CONCLUSION: Structural networks in patients SLE may be altered at both global and local levels, with more pronounced changes observed in NPSLE, notably affecting the default mode and sensorimotor networks. These alterations show promise as biomarkers for clinical diagnosis.
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Imagen de Difusión Tensora , Lupus Eritematoso Sistémico , Vasculitis por Lupus del Sistema Nervioso Central , Humanos , Femenino , Adulto , Masculino , Imagen de Difusión Tensora/métodos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/fisiopatología , Estudios Prospectivos , Vasculitis por Lupus del Sistema Nervioso Central/fisiopatología , Vasculitis por Lupus del Sistema Nervioso Central/psicología , Vasculitis por Lupus del Sistema Nervioso Central/diagnóstico , Vasculitis por Lupus del Sistema Nervioso Central/complicaciones , Persona de Mediana Edad , Estudios de Casos y Controles , Encéfalo/diagnóstico por imagen , Encéfalo/patologíaRESUMEN
OBJECTIVES: In childhood-onset SLE (cSLE), patients have an increased risk of premature atherosclerosis. The pathophysiological mechanisms for this premature atherosclerosis are not yet completely understood, but besides traditional risk factors, the endothelium plays a major role. The first aim of this study was to measure levels of SLE-associated markers involved in endothelial cell (EC) function and lipids in a cSLE cohort longitudinally in comparison with healthy controls (HC). Next aim was to correlate these levels with Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and nailfold capillaroscopic patterns. METHODS: Blood serum samples, videocapillaroscopy images and patient characteristics were collected in a multicentre longitudinal cSLE cohort and from age and sex comparable HC. Disease activity was evaluated by SLEDAI. A total of 15 EC markers and six lipids were measured in two longitudinal cSLE samples (minimum interval of 6 months) and in HC. Nailfold videocapillaroscopy images were scored according to the guidelines from the EULAR Study Group on Microcirculation in Rheumatic Diseases. RESULTS: In total, 47 patients with cSLE and 42 HCs were analysed. Median age at diagnosis was 15 years (IQR 12-16 years). Median time between t=1 and t=2 was 14.5 months (IQR 9-24 months). Median SLEDAI was 12 (IQR 6-18) at t=1 and 2 (IQR 1-4) at t=2. Serum levels of angiopoietin-2, CCL2, CXCL10, GAS6, pentraxin-3, thrombomodulin, VCAM-1 and vWF-A2 were elevated in cSLE compared with HC at t=1. While many elevated EC markers at t=1 normalised over time after treatment, several markers remained significantly increased compared with HC (angiopoietin-2, CCL2, CXCL10, GAS6, thrombomodulin and VCAM-1). CONCLUSION: In serum from patients with cSLE different markers of endothelial activation were dysregulated. While most markers normalised during treatment, others remained elevated in a subset of patients, even during low disease activity. These results suggest a role for the dysregulated endothelium in early and later phases of cSLE, possibly also during lower disease activity. TRIAL REGISTRATION NUMBER: NL60885.018.17.
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Biomarcadores , Lupus Eritematoso Sistémico , Angioscopía Microscópica , Humanos , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Femenino , Masculino , Niño , Adolescente , Biomarcadores/sangre , Estudios Longitudinales , Angioscopía Microscópica/métodos , Endotelio Vascular/fisiopatología , Edad de Inicio , Células Endoteliales , Índice de Severidad de la Enfermedad , Estudios de Casos y Controles , Trombomodulina/sangre , Lípidos/sangre , Aterosclerosis/sangre , Aterosclerosis/fisiopatologíaRESUMEN
BACKGROUND: Cognitive dysfunction (CD) is highly prevalent in systemic lupus erythematosus (SLE), yet the underlying mechanisms are poorly understood. Neuroimaging utilising advanced MRI metrics may yield mechanistic insights. We conducted a systematic review of neuroimaging studies to investigate the relationship between structural and diffusion MRI metrics and CD in SLE. METHODS: We systematically searched several databases between January 2000 and October 2023 according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Retrospective and prospective studies were screened for search criteria keywords (including structural or diffusion MRI, cognitive function and SLE) to identify peer-reviewed articles reporting advanced structural MRI metrics and evaluating CD in human patients with SLE. RESULTS: Eighteen studies (8 structural MRI, 9 diffusion MRI and 1 with both modalities) were included; sample sizes ranged from 11 to 120 participants with SLE. Neurocognitive assessments and neuroimaging techniques, parameters and processing differed across articles. The most frequently affected cognitive domains were memory, psychomotor speed and attention; while abnormal structural and/or diffusion MRI metrics were found more consistently in the hippocampus, corpus callosum and frontal cortex of patients with SLE, with and without clinically diagnosed central nervous system involvement. CONCLUSION: Advanced structural MRI analysis can identify total and regional brain abnormalities associated with CD in patients with SLE, with potential to enhance clinical assessment. Future collaborative, longitudinal studies of neuroimaging in SLE are needed to better characterise CD, with focus on harmonised neurocognitive assessments, neuroimaging acquisitions and postprocessing analyses and improved clinical characterisation of SLE cohorts.
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Encéfalo , Disfunción Cognitiva , Lupus Eritematoso Sistémico , Imagen por Resonancia Magnética , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/fisiopatología , Disfunción Cognitiva/etiología , Disfunción Cognitiva/fisiopatología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Imagen por Resonancia Magnética/métodos , Neuroimagen/métodos , Imagen de Difusión por Resonancia Magnética/métodos , Femenino , Masculino , Adulto , Pruebas NeuropsicológicasRESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease of undetermined etiology. Cardiac involvement is common in SLE and constitutes one of the main causes of mortality. More recently, new ultrasound imaging techniques, such as transthoracic ultrasound (TTE) with strain evaluation, have appeared and seem promising for the detection of cardiac involvement. The objective of our work was to study the frequency and characteristics of ultrasound abnormalities found in lupus patients and to study the benefit of ultrasound with global longitudinal strain (GLS) for early management. METHODS: It was an observational study of patients followed for SLE at the internal medicine and cardiology department of the HMPIT for 6 months (May-November 2023). The definition of cardiac involvement was by ultrasound. All patients benefited from TTE coupled with 2D-strain. We divided the workforce into two groups: the first group (patients with heart disease) and the second group (patients without heart disease). RESULTS: In a series of 40 lupus patients including 33 women and seven men, cardiac manifestations were reported in 60% of patients. In the first group, 29% had palpitations, 25% had chest pain, 67% had dyspnea, 37% had pericarditis, 8% had pulmonary arterial hypertension (PAH) and 12% had myocarditis. The comparative study showed that patients in the first group presented significantly more frequently with dyspnea (p = 0.02), chest pain (p = 0.03) and serositis (p = 0.01) compared to those in the second group. The mean left ventricular ejection fraction (LVEF) did not show a significant difference between the two groups. On the other hand, the average Global Longitudinal Strain (GLS) was significantly altered in the first group (p = 0.01). Furthermore, the frequency of pathological GLS was significantly higher in patients with lupus heart disease (p < 0.01). CONCLUSION: Cardiac involvement during SLE is a frequent and most often asymptomatic complication. A systematic search for this impairment using a high-performance echocardiography examination, namely the 2D GLS, is essential for early treatment.
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Ecocardiografía , Lupus Eritematoso Sistémico , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Femenino , Masculino , Adulto , Persona de Mediana Edad , Cardiopatías/etiología , Cardiopatías/diagnóstico por imagen , Disnea/etiología , Volumen Sistólico , Tensión Longitudinal GlobalRESUMEN
BACKGROUND AND OBJECTIVES: This study aimed to assess the utility of Doppler echocardiography in evaluating left ventricular diastolic function, and prognosis in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: A total of 286 SLE patients were selected along with 100 age- and gender-matched healthy individuals who underwent physical examinations. Clinical baseline characteristics were collected. Various Doppler echocardiographic parameters were measured and analyzed, including left ventricular posterior wall thickness (LVPWT), interventricular septal diameter (IVSD), left ventricular mass (LVM), LVM index (LVMI), and others. RESULTS: Compared to the control group, SLE patients exhibited significantly higher levels of C-reactive protein and lower levels of complement (C) 3 and C4 (p < .001). Doppler echocardiographic parameters showed significant differences between SLE patients and healthy controls, including increased LVPWT, IVSD, LVM, LVMI, peak A, PWI + Tei, E/e', TDI-Tei, and decreased e' and E/A (p < .001). Subgroup analyses indicated more severe ventricular diastolic dysfunction in patients with higher SLE activity and those who experienced cardiovascular events. Correlation analysis revealed positive associations of PWI + Tei, TDI-Tei, and GLS with SLE activity and cardiovascular events (p < .01). Multivariate logistic regression analysis identified LVMI, PWI + Tei, TDI-Tei, and GLS as significant predictors of cardiovascular events (p < .05). CONCLUSION: Doppler echocardiography is a valuable tool for the early diagnosis of left ventricular diastolic dysfunction in SLE patients. Key echocardiographic parameters, including LVMI, PWI + Tei, TDI-Tei, and GLS, are effective in predicting cardiovascular events, underscoring the importance of comprehensive cardiac function assessments in these patients.
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Ecocardiografía Doppler , Lupus Eritematoso Sistémico , Valor Predictivo de las Pruebas , Disfunción Ventricular Izquierda , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Lupus Eritematoso Sistémico/diagnóstico por imagen , Femenino , Masculino , Adulto , Ecocardiografía Doppler/métodos , Persona de Mediana Edad , Estudios de Casos y Controles , Pronóstico , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Función Ventricular Izquierda , Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/diagnóstico por imagen , Proteína C-Reactiva/análisis , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , DiástoleRESUMEN
BACKGROUND AND OBJECTIVES: Systemic Lupus Erythematosus (SLE) often causes damage to small nerve fibers, leading to distressing painful and autonomic symptoms. Despite this, Small Fiber Neuropathy (SFN) remains an underrecognized complication for SLE patients. In this cross-sectional study, we aimed to assess SFN in patients with SLE and to explore its correlations with immunologic disease features and clinical manifestations. METHODS: We recruited 50 SLE patients (1 male to 12.5 females, aged 20-80 years) reporting painful disturbances. We conducted a comprehensive clinical and neurophysiological evaluation, using Nerve Conduction Studies and Quantitative Sensory Testing. Additionally, we carried out an extensive laboratory assessment of disease-related serological parameters. We also performed a thorough skin biopsy analysis, investigating somatic and autonomic innervation while detecting complement and inflammatory cell infiltrates within the skin. RESULTS: Out of 50 patients, 19 were diagnosed with SFN, primarily characterized by a non-length-dependent distribution; 7 had a mixed neuropathy, with both large and small fiber involvement. Patients with SFN were younger than patients with a mixed neuropathy (p = .0143); furthermore, they were more likely to have a history of hypocomplementemia (p = .0058) and to be treated with cyclosporine A (p = .0053) compared to patients without neuropathy. However, there were no significant differences in painful and autonomic symptoms between patients with and without SFN. DISCUSSION: This study highlights the relevant frequency of SFN with a non-length-dependent distribution among SLE patients experiencing painful symptoms. Indeed, SFN emerges as an early manifestation of SLE-related neuropathy and is closely associated with hypocomplementemia, suggesting a potential pathogenic role of the complement system. Moreover, SFN may be influenced by disease-modifying therapies. However, the precise role of SFN in shaping painful and autonomic symptoms in patients with SLE remains to be fully elucidated.
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Lupus Eritematoso Sistémico , Neuropatía de Fibras Pequeñas , Humanos , Femenino , Masculino , Adulto , Persona de Mediana Edad , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/fisiopatología , Anciano , Neuropatía de Fibras Pequeñas/etiología , Neuropatía de Fibras Pequeñas/fisiopatología , Neuropatía de Fibras Pequeñas/diagnóstico , Neuropatía de Fibras Pequeñas/patología , Adulto Joven , Estudios Transversales , Anciano de 80 o más Años , Conducción Nerviosa/fisiología , Piel/patología , Piel/inervaciónRESUMEN
OBJECTIVE: To assess the impact of different disease activity patterns-long quiescent (LQ), chronically active (CA) and relapsing-remitting (RR)-on health-related quality of life (HRQoL) in a cohort of patients with systemic lupus erythematosus (SLE). METHODS: A retrospective, monocentric analysis of prospectively collected data. Adult SLE outpatients were enrolled between 2017 and 2021.For each year of follow-up, three disease activity patterns were defined: LQ if at each visit clinical Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Activity Index (SELENA-SLEDAI)=0, Physician Global Assessment (PGA)=0; CA if at each visit clinical SELENA-SLEDAI >0, PGA >0; RR if patients presented active disease in at least one visit during the observation period, interspersed with periods of remission. These patterns were applied to the year and the 3 years before enrolment.At enrolment, each patient completed: Short Form 36 (SF-36), Lupus Impact Tracker, Functional Assessment of Chronic Illness Therapy (FACIT), Hospital Anxiety and Depression Scale (HADS). The correlation between disease patterns and Patient-Reported Outcomes was analysed. RESULTS: 241 SLE patients were enrolled, of which 222 had complete clinical data for the 3-year period before enrolment. Both in the year and during the 3 years before enrolment, the most frequent disease pattern was the LQ (154/241 and 122/222 patients, respectively), followed by RR (53/241 and 92/222 patients, respectively) and CA (34/241 and 8/222 patients, respectively).At baseline, fibromyalgia, organ damage, age and daily glucocorticoid dose were associated with worse HRQoL.At the multivariable analysis, after adjusting for confounding factors, patients with LQ disease during the 3 years before enrolment presented a better physical HRQoL (SF-36 physical component summary, regression coefficient=3.2, 95% CI 0.51-5.89, p=0.02) and minor depressive symptoms (HADS-D, regression coefficient=-1.17, 95% CI -2.38 to 0.0.27, p=0.055), compared with patients with CA/RR disease. CONCLUSION: A persistently quiescent disease may have a positive impact on patients' physical HRQoL and on depressive symptoms. However, this condition appears insufficient to obtain a significant improvement in mental health, fatigue and disease burden among patients with SLE.
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Lupus Eritematoso Sistémico , Calidad de Vida , Humanos , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Calidad de Vida/psicología , Femenino , Adulto , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Medición de Resultados Informados por el Paciente , Depresión/psicología , Depresión/epidemiologíaRESUMEN
Systemic Lupus Erythematosus (SLE) is a chronic, autoimmune and multisystemic rheumatic disease. Patients with SLE have decreased functional and aerobic capacity, as well as increased prevalence of Cardiovascular Diseases (CVD), which are the primary causes of morbimortality in this condition. Dietary intake and physical activity are well-known modifiable cardiovascular risk factors. The aim of this study is to describe food consumption, sedentary behavior, physical activity level, and functional and aerobic capacity in a sample of SLE patients with high cardiovascular risk. This was a cross-sectional study in which patients were assessed for (i) Demographic, anthropometric, and disease-related parameters; (ii) Food consumption; (iii) Physical activity level and sedentary behavior; (iv) Functional and aerobic capacity. Patients averaged 41.7 ± 9 years, and most were classified as overweight/obese (87%). Average macronutrient intake was within recommendations; however, fiber (16 ± 9g) and calcium (391 ± 217 mg) intakes were below, and sodium intake (2.9 ± 1.3 mg) was above recommendations. Besides, food consumption assessed by the Nova system showed a predominance of unprocessed foods (43.8 ± 14.0%TEI), although ultraprocessed food intake (20.0 ± 13.9%TEI) was slightly higher than that seen in the Brazilian population. Patients also exhibited high sedentary behavior (8.2 ± 2.2h) and only eighteen participants reached the minimum recommended amount of moderate-to-vigorous physical activity. Overall, patients had a low functional and aerobic capacity compared to the general population. Data from this study may help design dedicated clinical trials aiming to investigate the effects of lifestyle intervention to mitigate CVD in SLE.
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Enfermedades Cardiovasculares , Ejercicio Físico , Factores de Riesgo de Enfermedad Cardiaca , Lupus Eritematoso Sistémico , Conducta Sedentaria , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Femenino , Estudios Transversales , Adulto , Ejercicio Físico/fisiología , Masculino , Persona de Mediana Edad , Enfermedades Cardiovasculares/etiología , Brasil/epidemiología , Conducta Alimentaria/fisiología , Factores de Riesgo , Ingestión de Alimentos/fisiología , Índice de Masa CorporalRESUMEN
OBJECTIVE: To determine if self-reported fatigue, anxiety, depression, cognitive difficulties, health-related quality of life, disease activity scores and neuropsychological battery (NB) cluster into distinct groups in patients with SLE based on symptom intensity and if they change at 1-year follow-up. METHODS: This is a retrospective analysis of consecutive consenting patients, followed at a single centre. Patients completed a comprehensive NB, the Beck Anxiety Inventory, Beck Depression Inventory, Fatigue Severity Scale, Short-Form Health Survey Physical Component Summary and Mental Component Summary scores and the Perceived Deficits Questionnaire. Disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index 2000. Ward's method was used for clustering and principal component analysis was used to visualise the number of clusters. Stability at 1 year was assessed with kappa statistic. RESULTS: Among 142 patients, three clusters were found: cluster 1 had mild symptom intensity, cluster 2 had moderate symptom intensity and cluster 3 had severe symptom intensity. At 1-year follow-up, 49% of patients remained in their baseline cluster. The mild cluster had the highest stability (77% of patients stayed in the same cluster), followed by the severe cluster (51%), and moderate cluster had the lowest stability (3%). A minority of patients from mild cluster moved to severe cluster (19%). In severe cluster, a larger number moved to moderate cluster (40%) and fewer to mild cluster (9%). CONCLUSION: Three distinct clusters of symptom intensity were documented in patients with SLE in association with cognitive function. There was a lower tendency for patients in the mild and severe clusters to move but not moderate cluster over the course of a year. This may demonstrate an opportunity for intervention to have moderate cluster patients move to mild cluster instead of moving to severe cluster. Further studies are necessary to assess factors that affect movement into moderate cluster.
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Cognición , Lupus Eritematoso Sistémico , Calidad de Vida , Autoinforme , Índice de Severidad de la Enfermedad , Humanos , Femenino , Masculino , Calidad de Vida/psicología , Adulto , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Persona de Mediana Edad , Estudios Retrospectivos , Cognición/fisiología , Análisis por Conglomerados , Fatiga/psicología , Fatiga/epidemiología , Depresión/epidemiología , Depresión/psicología , Afecto , Ansiedad/epidemiología , Ansiedad/psicología , Pruebas Neuropsicológicas/estadística & datos numéricos , Estudios de Seguimiento , Encuestas y CuestionariosRESUMEN
OBJECTIVES: This study aimed to evaluate the clinical value of the Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) for assessing disease activity in patients with SLE. METHODS: Clinical data were collected from patients with SLE who were admitted at the Second Affiliated Hospital of Soochow University from January 2009 to December 2022. The glucocorticoid dose grading was used as the gold standard for disease activity assessment in SLE. The SLE-DAS value was calculated, and the SLE disease activity status was graded based on the SLE-DAS value. Another scoring criterion, the SLE Disease Activity Index 2000 (SLEDAI 2000), served as a control. Spearman correlation analysis was used to calculate the correlation between the scoring criteria and other variables. RESULTS: The analysis included 396 patients with SLE. A strong correlation was found between SLE-DAS and SLEDAI 2000 (ρ=0.709, 95% CI 0.648 to 0.766, p<0.001), with median SLE-DAS and SLEDAI 2000 scores of 15.32 (7.90 to 24.45) and 13 (8 to 19), respectively. Compared with the SLEDAI 2000 value, the SLE-DAS value correlated better with glucocorticoid dose grading (ρ=0.434 vs 0.518), gammaglobulin use (ρ=0.170 vs 0.318) and immunosuppressant use (ρ=0.122 vs 0.221). A moderate correlation based on disease activity grading was found between SLE-DAS and glucocorticoid dose grading (ρ=0.441), whereas a mild correlation was observed between SLEDAI 2000 and glucocorticoid dose grading (ρ=0.325). Additionally, SLE-DAS revealed a positive correlation with severe thrombocytopenia, cardiac involvement and pulmonary involvement but not SLEDAI 2000. CONCLUSION: Compared with SLEDAI 2000, SLE-DAS may provide a more accurate disease activity assessment in patients with SLE, especially those with severe thrombocytopenia and cardiopulmonary involvement.
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Glucocorticoides , Lupus Eritematoso Sistémico , Índice de Severidad de la Enfermedad , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/fisiopatología , Femenino , Masculino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Glucocorticoides/uso terapéutico , Adulto JovenRESUMEN
OBJECTIVE: In this pilot study, we used untargeted metabolomics to identify biochemical mechanisms or biomarkers potentially underlying SLE-related fatigue. METHODS: Metabolon conducted untargeted metabolomic plasma profiling using ultrahigh performance liquid chromatography/tandem mass spectrometry on plasma samples of 23 Black females with systemic lupus erythematosus (SLE) and 21 no SLE controls. Fatigue phenotypes of general fatigue, physical fatigue, mental fatigue, reduced activity, and reduced motivation were measured with the reliable and valid Multidimensional Fatigue Inventory (MFI). RESULTS: A total of 290 metabolites were significantly different between the SLE and no SLE groups, encompassing metabolites related to glycolysis, TCA cycle activity, heme catabolism, branched chain amino acids, fatty acid metabolism, and steroids. Within the SLE group, controlling for age and co-morbidities, TCA cycle metabolites of alpha-ketoglutarate (AKG) and succinate were statistically significantly associated (p < .05) with physical and general fatigue. CONCLUSION: While pervasive perturbations in the entire TCA cycle have been implicated as a potential mechanism for fatigue, our results suggest individual metabolites of AKG and succinate may be potential biomarkers or targets of intervention for fatigue symptom management in SLE. Additionally, perturbations in heme metabolism in the SLE group provide additional insights into mechanisms that promote systemic inflammation.
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Biomarcadores , Ciclo del Ácido Cítrico , Fatiga , Lupus Eritematoso Sistémico , Metabolómica , Humanos , Femenino , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Proyectos Piloto , Fatiga/etiología , Fatiga/sangre , Adulto , Metabolómica/métodos , Biomarcadores/sangre , Persona de Mediana Edad , Negro o Afroamericano , Espectrometría de Masas en Tándem , Estudios de Casos y Controles , Ácido Succínico/sangre , Ácidos Cetoglutáricos/sangre , Cromatografía Líquida de Alta PresiónRESUMEN
OBJECTIVE: To evaluate the predictive value of the LFA-REAL ClinRO (Lupus Foundation of America Rapid Evaluation of Activity in Lupus clinician-reported outcome) on damage accrual in systemic lupus erythematosus patients. METHODS: Data from a prevalent lupus cohort were used. The LFA-REAL ClinRO includes 9 domains: mucocutaneous (global and 3 subdomains), musculoskeletal (global and 2 subdomains), cardiorespiratory, neuropsychiatric, renal, hematological, constitutional, vasculitis, and other (it allows for other or rare manifestations). For each domain, a 0- to 100-mm visual analog scale is used, and global domains are included except for the mucocutaneous and musculoskeletal domains where the subdomains are included; it allows for 3 manifestations under "other," so the score ranges from 0 to 1400 (sum of 14 in the visual analog scale). Damage was assessed with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index. Generalized estimating equations were performed, being the outcome the increase in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index; confounders from the previous visit were included; adjusted multivariable models were done. Incidence rate ratios per 10-unit increase in the LFA-REAL ClinRO were reported. Similar models were performed to evaluate the impact of the SLEDAI-2K (SLE Disease Activity Index) and physician global assessment on damage to determine which measure would better predict damage accrual. RESULTS: Three-hundred thirty-one patients and 1425 visits were included, 1.9 (SD 1.2) years of follow-up. Disease duration at baseline was 10.7 (7.4) years. The mean LFA-REAL ClinRO was 18.2 (SD 30.7). During the follow-up visits, 63 (17.9%) patients accrued damage once; 4 (1.1%) accrued damage twice. The LFA-REAL ClinRO was predictive of damage accrual even after adjustment for possible confounders (incidence rate ratio 1.10 (95% confidence interval 1.04-1.16; p < 0.001). Similar results were obtained using the SLEDAI-2K and the physician global assessment. CONCLUSION: The LFA-REAL ClinRO is predictive of damage accrual, even after adjusting for possible confounders.
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Lupus Eritematoso Sistémico , Índice de Severidad de la Enfermedad , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/fisiopatología , Lupus Eritematoso Sistémico/epidemiología , Femenino , Masculino , Adulto , Persona de Mediana Edad , Estudios de Cohortes , Valor Predictivo de las Pruebas , Progresión de la EnfermedadRESUMEN
Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease associated with an increased risk of cardiovascular diseases (CVDs), leading to elevated mortality rates among patients. We aimed to evaluate the levels of cardio-ankle vascular index (CAVI), global longitudinal strain (GLS), ventricular-arterial coupling (VAC), and high-sensitivity cardiac troponin I (hsTnI) in SLE patients and to explore their relationship with clinical parameters. Methods: This cross-sectional study enrolled 82 SLE patients without evident cardiac or kidney impairment and 41 age- and sex-matched healthy controls. We comparatively evaluated CAVI, GLS, VAC, and hsTnI between SLE patients and controls, and we assessed their association among SLE patients with disease activity based on the SELENA-SLEDAI Activity Index. Multivariate regression analysis was performed to identify independent predictors of CAVI and hsTnI within the SLE cohort. Results: In comparison to healthy controls, SLE patients presented with significantly higher CAVI, GLS, and hsTnI levels, while VAC was significantly reduced (p < 0.001). Furthermore, SLE patients with active disease (SELENA-SLEDAI ≥ 4) exhibited higher levels of CAVI and troponin than those with inactive disease (p < 0.001). SLEDAI was an independent predictor of CAVI, while VAC and SLEDAI were independent determinants of hsTnI in the SLE cohort. Conclusions: SLE patients displayed abnormal levels of CAVI, VAC, GLS, and troponin compared to healthy individuals. Our findings implicate the potential of those CV novel CVD risk factors to refine screening and therapeutic strategies for this specific population.
Asunto(s)
Enfermedades Cardiovasculares , Lupus Eritematoso Sistémico , Troponina I , Rigidez Vascular , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Biomarcadores/sangre , Índice Vascular Cardio-Tobillo , Enfermedades Cardiovasculares/sangre , Enfermedades Cardiovasculares/fisiopatología , Enfermedades Cardiovasculares/etiología , Estudios de Casos y Controles , Estudios Transversales , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/fisiopatología , Troponina I/sangre , Rigidez Vascular/fisiologíaRESUMEN
OBJECTIVE: to learn the meanings attributed to pregnancy loss by women with Lupus. METHOD: qualitative research, based on Symbolic Interactionism and Grounded Theory. Data collection took place between January and August 2022 through in-depth interviews. Data analysis went through the stages of initial and focused coding. RESULTS: seventeen women participated. The central phenomenon "The climb to motherhood: falls and overcoming" was constructed, consisting of three categories: "Falling to the ground during the climb: the experience of pregnancy loss"; "Getting up and following the path: new attempts to conceive"; and "Remembering the journey: meanings attributed to pregnancy losses". FINAL CONSIDERATIONS: experiencing pregnancy is, analogously, like climbing a mountain, where obstacles need to be overcome to reach the summit. The experience of pregnancy loss is seen as complex, especially when there is fragility in healthcare and a lack of awareness regarding feelings of loss and grief.
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Aborto Espontáneo , Teoría Fundamentada , Lupus Eritematoso Sistémico , Investigación Cualitativa , Humanos , Femenino , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Embarazo , Adulto , Aborto Espontáneo/psicología , Entrevistas como Asunto/métodosRESUMEN
OBJECTIVE: This study aimed to evaluate the prevalence of sarcopenia and its clinical significance in Turkish women with SLE, exploring the association between muscle mass, muscle strength and SLE disease activity. METHODS: A cross-sectional study was conducted at Gazi University Hospital's Department of Rheumatology from January to December 2020. It involved 82 patients with SLE, diagnosed according to the 2019 American College of Rheumatology/European Alliance of Associations for Rheumatology criteria, and 69 healthy controls. Sarcopenia was assessed using hand grip dynamometry (hand grip strength (HGS)) and bioelectrical impedance analysis for muscle mass, with sarcopenia defined according to the 2018 European Working Group on Sarcopenia in Older People criteria and specific cut-offs for the Turkish population. The main outcomes measured were the presence of sarcopenia and probable sarcopenia, HGS values, skeletal muscle mass index and SLE Disease Activity Index 2000 (SLEDAI-2K). RESULTS: Among the patients with SLE, 51.2% met the criteria for probable sarcopenia and 12.9% were diagnosed with sarcopenia. The mean HGS was significantly lower in the SLE group (21.7±4.9 kg) compared with controls, indicating reduced muscle strength. The prevalence of anti-double-stranded DNA (anti-dsDNA) antibodies was 82.9%. Multivariate regression analysis identified height and levels of anti-dsDNA antibodies as independent predictors for developing probable sarcopenia. No significant association was found between clinical parameters, including SLEDAI-2K scores, and sarcopenia status. CONCLUSIONS: Sarcopenia is prevalent among Turkish women with SLE, with a significant proportion showing reduced muscle strength. The study found no direct association between sarcopenia and SLE disease activity or clinical parameters. These findings underscore the importance of including muscle strength assessments in the routine clinical evaluation of patients with SLE to potentially improve management and quality of life.
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Fuerza de la Mano , Lupus Eritematoso Sistémico , Fuerza Muscular , Sarcopenia , Humanos , Sarcopenia/epidemiología , Sarcopenia/fisiopatología , Sarcopenia/diagnóstico , Femenino , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/fisiopatología , Estudios Transversales , Turquía/epidemiología , Adulto , Persona de Mediana Edad , Prevalencia , Estudios de Casos y Controles , Anticuerpos Antinucleares/sangre , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Índice de Severidad de la EnfermedadRESUMEN
INTRODUCTION: Sexual dysfunction (SD) is highly prevalent and multifactorial; nevertheless, recent research has shed light on a notable phenomenon: male patients with systemic lupus erythematosus (SLE) exhibit an elevated prevalence of sexual function disorders compared with the general population. Despite this recognition, the precise nature and extent of this association remain incompletely understood. OBJECTIVES: This comprehensive review aims to clarify the link by providing an overview of the fundamental components of normal male sexual function, delving into the pathogenesis of male SD and exploring the primary factors predisposing male SLE patients to SD. Additionally, the review offers insights into potential screening, diagnostic, and treatment strategies based on the current body of literature. METHODS: A meticulous search of relevant literature was conducted using the PubMed and Google Scholar databases. RESULTS: Studies exploring the correlation between SLE and SD in both genders have revealed a nearly 2-fold increased risk of SD among individuals with SLE compared with healthy counterparts. Moreover, these studies suggest that male SLE patients may have a higher susceptibility to SD, with reported prevalence ranging from 12% to 68%, compared with 0% to 22% in healthy individuals. Male patients with SLE are influenced by a spectrum of pathological factors, including pharmacological, psychological, and disease-related determinants, which, through their intricate interplay, elevate the likelihood of developing SD. CONCLUSION: Healthcare professionals must remain vigilant in understanding the intricacies of human sexuality and its dysfunction, particularly in males with SLE. The objective is to establish effective and potentially standardized methods for promptly diagnosing and optimally managing SD, recognizing its significant impact on the quality of life for males living with SLE. The pivotal role of rheumatologists in initiating discussions about sexual health, diagnosing SD, investigating causes, and implementing tailored strategies is underscored as crucial in addressing this multifaceted issue.
Asunto(s)
Lupus Eritematoso Sistémico , Disfunciones Sexuales Fisiológicas , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Disfunciones Sexuales Fisiológicas/etiología , Reumatólogos , PrevalenciaRESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease. Cardiac involvement in SLE is rare but plays an important prognostic role. The degree of cardiac involvement according to SLE subsets defined by non-cardiac manifestations is unknown. The objective of this study was to identify differences in transthoracic echocardiography (TTE) parameters associated with different SLE subgroups. METHODS: One hundred eighty-one patients who fulfilled the 2019 American College of Rheumatology/EULAR classification criteria for SLE and underwent baseline TTE were included in this cross-sectional study. We defined four subsets of SLE based on the predominant clinical manifestations. A multivariate multinomial regression analysis was performed to determine whether TTE parameters differed between groups. RESULTS: Four clinical subsets were defined according to non-cardiac clinical manifestations: group A (n=37 patients) showed features of mixed connective tissue disease, group B (n=76 patients) had primarily cutaneous involvement, group C (n=18) exhibited prominent serositis and group D (n=50) had severe, multi-organ involvement, including notable renal disease. Forty TTE parameters were assessed between groups. Per multivariate multinomial regression analysis, there were statistically significant differences in early diastolic tricuspid annular velocity (RV-Ea, p<0.0001), RV S' wave (p=0.0031) and RV end-diastolic diameter (p=0.0419) between the groups. Group B (primarily cutaneous involvement) had the lowest degree of RV dysfunction. CONCLUSION: When defining clinical phenotypes of SLE based on organ involvement, we found four distinct subgroups which showed notable differences in RV function on TTE. Risk-stratifying patients by clinical phenotype could help better tailor cardiac follow-up in this population.
Asunto(s)
Ecocardiografía , Ventrículos Cardíacos , Lupus Eritematoso Sistémico , Función Ventricular Derecha , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/fisiopatología , Femenino , Masculino , Estudios Transversales , Adulto , Persona de Mediana Edad , Función Ventricular Derecha/fisiología , Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/etiología , Disfunción Ventricular Derecha/diagnóstico por imagen , Estudios Retrospectivos , PronósticoRESUMEN
OBJECTIVES: Substantial proportions of patients with SLE report poor health-related quality of life (HRQoL). Our objective was to investigate the impact of neuropsychiatric involvement (NP) in SLE on patient-reported outcomes. METHODS: We analysed data from four phase III trials (BLISS-52, BLISS-76, BLISS-SC, EMBRACE; N = 2968). The NPSLE group comprised individuals with NP-BILAG A/B/C/D or score in any descriptor of the NP-SLEDAI-2K at baseline (N = 350), while the non-NPSLE group consisted of patients with NP-BILAG E (N = 2618). HRQoL was assessed with the SF-36, EQ-5D-3L, and FACIT-F. Full health state (FHS) was defined as 'no problems' in all EQ-5D dimensions. RESULTS: NPSLE patients reported lower scores in the SF-36 physical and mental component summary compared with the non-NPSLE population [mean (s.d.): 35.7 (9.1) vs 39.6 (9.6); P < 0.001 and 37.3 (12.1) vs 41.4 (11.0); P < 0.001, respectively]. NPSLE patients also exhibited impaired HRQoL in all EQ-5D dimensions compared with non-NPSLE patients (P < 0.05 for all). A substantially lower proportion of NPSLE patients experienced FHS in comparison with the non-NPSLE group (3.3% vs 14.5%; P < 0.001). NPSLE was associated with severe fatigue [23.8 (12.2) vs 31.5 (11.6); P < 0.001]. Notably, our findings revealed no discernible distinctions between active and inactive NPSLE patients with regard to SF-36, EQ-5D, FHS or FACIT-F scores. CONCLUSION: NP in patients with SLE has a detrimental effect on HRQoL experience and is associated with severe fatigue, regardless of the degree of neuropsychiatric disease activity. Early intervention is warranted in NPSLE patients to enhance long-term HRQoL experience.
Asunto(s)
Fatiga , Lupus Eritematoso Sistémico , Calidad de Vida , Humanos , Femenino , Masculino , Fatiga/etiología , Fatiga/psicología , Fatiga/fisiopatología , Adulto , Persona de Mediana Edad , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/fisiopatología , Medición de Resultados Informados por el Paciente , Índice de Severidad de la Enfermedad , Vasculitis por Lupus del Sistema Nervioso Central/psicología , Vasculitis por Lupus del Sistema Nervioso Central/fisiopatología , Estado de SaludRESUMEN
OBJECTIVE: Constitutional symptoms (fatigue, lymphadenopathy, and weight loss) are not included in the SLE disease activity index-2000 (SLEDAI-2K). In this pilot study, we assessed the concurrent and construct validity of a revised SLEDAI-2K (SLED-R) that included these symptoms with the original SLEDAI-2K (SLED-O), using the physician global assessment of disease activity (PGA) as the reference. METHODS: Our revised SLED-R substituted the SLED-O's fever descriptor with a constitutional descriptor that included fever, fatigue, lymphadenopathy, and/or weight loss. SLED-O, SLED-R, PGA and patient global assessment (PtGA) scores were collected prospectively. Bland-Altman correlations for repeated measures were calculated and Meng's z-test was used to compare correlations between dependent and overlapping correlation coefficients. Associations between constitutional symptoms and disease activity measures were analyzed using Mann-Whitney U, Kruskal-Wallis, Chi-square tests and repeated measures correlations. RESULTS: 1123 SLED-O, SLED-R, PGA, and 1066 PtGA were collected in 239 subjects. The new descriptor was scored in 45 subjects (18.8%) and 92 instances (8.1%), while the original descriptor, fever, was scored in only 4 subjects (1.7%) and 5 instances (0.4%). Mean SLED-O, PGA and PtGA scores were higher when the constitutional descriptor was scored versus not (p < .001). The correlation between SLED-R and PGA was marginally higher than between SLED-O and PGA (p < .001). Fatigue contributed most to this increase (p = .001) and associated with both higher PGA and PtGA scores (p < .001). Mean SLED-O and PGA scores were higher when ≥1 constitutional symptom(s) were scored versus not (p < .002). Correlations between PGA and PtGA when the new descriptor was scored versus not were similar (p = .860). The frequency of concordance between PGA and PtGA was lower when the new descriptor was scored (55%) versus not (72.5%), with PGA > PtGA when the new descriptor was scored (p < .001). CONCLUSION: The addition of constitutional symptoms to SLEDAI-2K, particularly fatigue, resulted in a marginal increase in its correlation with PGA, and new constitutional symptoms associated with higher SLED-O and PGA scores. As fatigue is subjective and difficult to attribute to SLE, its validity and inter-rater reliability in scoring remains uncertain. The clinical utility of SLED-R remains unclear, and further studies of its validity and reliability are needed.