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1.
Clin Exp Rheumatol ; 42(5): 1104-1114, 2024 05.
Artículo en Inglés | MEDLINE | ID: mdl-38743446

RESUMEN

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. SLE pathogenesis is the result of complex interactions between ethnic, genetic, epigenetic, immunoregulatory, hormonal and environmental factors, and several aspects of these multifactorial connections are still unclear. Overall, for the disease development, an environmental trigger may induce immunological dysfunction in genetically predisposed individuals. This review aims to summarise the most relevant data on the impact of environmental factors on the incidence of SLE and on disease activity and damage in patients with an established diagnosis of SLE.


Asunto(s)
Interacción Gen-Ambiente , Lupus Eritematoso Sistémico , Humanos , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/genética , Lupus Eritematoso Sistémico/diagnóstico , Factores de Riesgo , Predisposición Genética a la Enfermedad , Incidencia , Exposición a Riesgos Ambientales/efectos adversos , Ambiente
2.
RMD Open ; 10(2)2024 Apr 12.
Artículo en Inglés | MEDLINE | ID: mdl-38609320

RESUMEN

OBJECTIVE: The objective is to evaluate perscriptions of belimumab (BEL), how these have changed over the years and their impact on clinical outcomes in patients with systemic lupus erythematosus (SLE). METHODS: This is a retrospective analysis of prospectively collected data. We retrieved demographic and clinical data and concomitant therapies at BEL starting (baseline). Disease activity was assessed at baseline and after 6 and 12 months and organ damage at baseline and at the last visit. RESULTS: From 422 patients followed in the Pisa SLE cohort, 102 patients received BEL and were included and 22 (21.6%) were immunosuppressant (IS)-naïve. Lupus Low Disease Activity State (LLDAS) with a glucocorticoid (GC) dosage ≤5 mg/day (LLDAS5) and remission were achieved by 47% and 38% of patients at 6 months, and by 75% and 66% at 12 months. Comparing IS-naïve patients with those who received BEL after at least one conventional IS, we did not find significant differences in baseline characteristics and in the achievement of LLDAS5 and remission. Despite at baseline we did not observe significant differences in mean GC daily dosage, IS-naïve patients were taking a significantly lower GC daily dose at 6 and 12 months. Interestingly, IS-naïve patients were more common in the most recent years. CONCLUSIONS: Our data confirm that BEL is effective in controlling disease activity, and in recent years BEL has been considered as an earlier treatment option before other IS. Early introduction of BEL can be at least as effective as a step-up approach and can help to reduce the GC dosage.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Lupus Eritematoso Sistémico , Humanos , Estudios Retrospectivos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Prescripciones
3.
RMD Open ; 10(2)2024 Apr 25.
Artículo en Inglés | MEDLINE | ID: mdl-38670557

RESUMEN

OBJECTIVES: Undifferentiated connective tissue diseases (UCTDs) are systemic autoimmune conditions that cannot be diagnosed nor classified as defined CTD; the majority maintains an undifferentiated profile (stable UCTD, sUCTD) over time. Data on long-term outcomes of sUCTD are lacking. METHODS: Retrospective longitudinal analysis of an inception cohort of 141 patients with sUCTD.Disease evolution and damage accrual were evaluated at 1, 5 and 10 years. Partial least square (PLS) regression was used to identify the basal variables contributing to damage accrual at 1, 5 and 10 years of follow-up. Trend of damage over time was compared with a cohort of age-matched and sex-matched patients with systemic lupus erythematosus (SLE) by means of Nelson-Aalen analysis. RESULTS: 11.3% of patients evolved to a definite CTD after a median 11 years (IQR 6-25) from the first symptom. At last visit, 10% were on glucocorticoids and 6% on immunosuppressive therapy. In 27.3%, at least one item of organ damage was recorded according to the SLICC/DI score (mean score 1.19±0.46). At PLS analysis, age at diagnosis and age at first symptoms were related to damage at 1 year, not taking antimalarials and taking immunosuppressants were associated with damage at 5 years.The mean survival without damage was 9.3 years in sUCTD and 8.4 years in SLE. The 10-year probability without damage was 62% and 23% in SLE and sUCTD, respectively (p=0.015). CONCLUSIONS: Although less significantly impacted than in patients with SLE, in the long-term UCTDs can accumulate organ damage and evolve into defined connective tissue diseases.


Asunto(s)
Progresión de la Enfermedad , Lupus Eritematoso Sistémico , Enfermedades Indiferenciadas del Tejido Conectivo , Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Enfermedades Indiferenciadas del Tejido Conectivo/complicaciones , Enfermedades Indiferenciadas del Tejido Conectivo/epidemiología , Enfermedades Indiferenciadas del Tejido Conectivo/diagnóstico , Estudios Longitudinales , Inmunosupresores/uso terapéutico , Índice de Severidad de la Enfermedad , Glucocorticoides/uso terapéutico
4.
Clin Exp Rheumatol ; 42(3): 583-592, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38545801

RESUMEN

Systemic lupus erythematosus (SLE) is classically regarded as the landmark of systemic autoimmune diseases, characterised by protean, multi-systemic manifestations and a highly variable clinical course.Over the last years, both clinical and translational clinical research efforts led to significant steps forward in management and treatment of SLE. However, numerous aspects of SLE, from pathogenesis to treatment, still remain challenging, and several unmet needs persist for both patients and physicians. Following the previous annual reviews of this series, herewith, we aim to report the most relevant new updates on SLE, issued in 2023. In particular, we focused on biomarkers, clinical aspects and outcomes, comorbidities, as well as new treatment targets and real-world evidence.


Asunto(s)
Lupus Eritematoso Sistémico , Médicos , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Biomarcadores , Comorbilidad
5.
Rheumatology (Oxford) ; 63(2): 376-384, 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-37094218

RESUMEN

OBJECTIVES: To describe phenotypes and outcomes of extra-renal flares in SLE, to identify clusters of extra-renal flares based on baseline features, and to develop a machine learning (ML) tool capable of predicting 'difficult to treat' (D2T) flares. METHODS: Extra-renal flares that occurred in our cohort over the last five years with at least one year of follow-up were included. Baseline clinical variables were described and flares assigned to clusters. Attainment of remission and low disease activity state (LLDAS) at 12 months were compared. Flares were then considered 'D2T' in case of non-attainment of LLDAS at 6 and 12 months. Baseline features were used to train a ML model able to predict future D2T-flares, at admission. Traditional approaches were then compared with informatic techniques. RESULTS: Among 420 SLE patients of the cohort, 114 flares occurred between 2015 and 2021; 79 extra-renal flares, predominantly mucocutaneous (24.1%) and musculoskeletal (45.6%), were considered. After 12 months, 79.4% and 49.4% were in LLDAS and in remission, respectively, while 17 flares were classified as D2T (21.5%); D2T flares received a higher cumulative and daily dose of glucocorticoids. Among the clusters, cluster 'D' (mild-moderate flares with mucocutaneous manifestations in patients with history of skin involvement) was associated with the lowest rate of remission. Among clinical data, not being on LLDAS at 3 months was the unique independent predictor of D2T flares. CONCLUSIONS: Our clusterization well separates extra-renal flares according to their baseline features and may propose a new identification standard. D2T flares, especially refractory skin manifestations, are frequent in SLE and represent an unmet need in the management of the disease as they are associated with higher glucocorticoid (GC) dosage and risk of damage accrual. Our ML model could help in the early identification of D2T flares, flagging them to elevate the attention threshold at admission.


Asunto(s)
Lupus Eritematoso Sistémico , Humanos , Estudios Longitudinales , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/complicaciones , Glucocorticoides/uso terapéutico , Riñón , Medición de Riesgo , Índice de Severidad de la Enfermedad
6.
Clin Exp Rheumatol ; 41(5): 997-1008, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37133502

RESUMEN

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations and a relapsing-remitting course. New data regarding pathogenic pathways, biomarkers and clinical manifestations of SLE are emerging, and new drugs and therapeutic protocols have been proposed to improve the control of disease activity. Furthermore, new insights into comorbidities and reproductive health in SLE patients are constantly emerging.This annual review aims to summarise the most relevant data on SLE that was published in 2022.


Asunto(s)
Enfermedades Autoinmunes , Lupus Eritematoso Sistémico , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Comorbilidad , Biomarcadores/metabolismo
7.
Lupus Sci Med ; 10(1)2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-37185240

RESUMEN

BACKGROUND: SLE is an autoimmune disease that predominantly affects women. As most epidemiological and interventional studies are on populations with a clear female prevalence, the influence of gender in disease course, drug response and damage accrual is yet to be fully explored and comprehended. OBJECTIVES: To describe gender differences in disease course, comorbidities, use of medications and long-term outcomes of a large cohort of patients with SLE. METHODS: Retrospective gender-based analysis of prospectively collected data from a monocentric cohort of Caucasian patients with SLE with at least 1 year of follow-up. RESULTS: 417 patients were included, 51 men and 366 women. Men displayed a significantly higher median age at disease onset and diagnosis and a higher prevalence of late-onset SLE, serositis at disease onset, antiphospholipid syndrome (APS) and use of mycophenolate within the first year of disease. Women had a higher prevalence of haematological abnormalities, a higher cumulative exposure to azathioprine and higher cumulative dose of glucocorticoids at 5 years. Male patients had a shorter time to first damage item and a higher prevalence of damage at 1 and 5 years, but this association was no longer significant when late-onset patients were excluded. No differences were found in prevalence of childhood onset, delay between onset and diagnosis, time to renal involvement and histology, cumulative autoantibody positivity, number of flares and hospitalisations, median SLE Damage Index score, type of damage, age and time to first cardiovascular event, chronic kidney disease and death. CONCLUSIONS: In our cohort, clinical manifestations and disease course were similar in male and female patients; however, male patients displayed higher prevalence of APS and early damage accrual probably due to the later disease onset. These data highlight the importance of an intensive follow-up, prevention and treatment of complications in this category of patients, especially in the first years of disease.


Asunto(s)
Síndrome Antifosfolípido , Lupus Eritematoso Sistémico , Humanos , Masculino , Femenino , Estudios Retrospectivos , Factores Sexuales , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Glucocorticoides/efectos adversos , Progresión de la Enfermedad
8.
Front Med (Lausanne) ; 9: 859840, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35425779

RESUMEN

Aims of the study: To analyze the prevalence of self-reported anxiety and depression in a monocentric cohort of patients with Systemic Lupus Erythematosus (SLE); to study the main determinants and the impact on quality of life (QoL). Methods: A cross-sectional observational study including adult outpatients with SLE. Demographic and clinical data were analyzed: indices of disease activity (SELENA-SLEDAI); damage (SLICC-DI); comorbidities and concomitant therapies. The definitions for remission (DORIS) and "Lupus Low Disease Activity State" (LLDAS) were applied. At enrollment, each patient completed the following questionnaires: SF-36, FACIT-Fatigue, Lupus Impact Tracker (LIT), Systemic Lupus Activity Questionnaire (SLAQ), and the Hospital Anxiety and Depression Scale (HADS) in order to self-assess anxiety and depression symptoms. The Student t-test and Chi2 tests were conducted for univariate analysis. The Spearman test was used for linear correlation between continuous data. Multivariate analysis was performed by multiple linear and logistic regression. Results: One hundred fifty-four consecutive patients with SLE were enrolled, the majority female and Caucasian with a mean age = 43.3 ± 13.7 years. 79.9% were in LLDAS or remission. 36.4% had a SDI > 1. 13.7% of patients had concomitant fibromyalgia. 37.4% had symptoms indicating anxiety and 25% of depression according to the HADS questionnaire. In the multivariate analysis, patients with active disease were significantly more anxious and depressed (p < 0.01) compared to patients in LLDAS or remission. Fibromyalgia and older age were independently associated with anxiety and depression, respectively (p < 0.05). Active skin involvement was significantly linked to depression (p < 0.05). Higher scores on the HADS questionnaire (higher levels of anxiety and depression) were found to be significantly linked to patients' perception of higher disease activity and worse quality of life, irrespective of disease activity, age and fibromyalgia. Conclusion: Symptoms of anxiety and depression are frequent in SLE patients, including outpatients with mild/moderate disease. Such symptoms have a significant negative impact on QoL and perception of disease activity, regardless of other factors. Moreover, disease activity, advanced age and fibromyalgia appear to be significantly linked to mood disorders. Assessing symptoms of the anxious-depressive spectrum in patients with SLE could lead to improvement in patients' perception of health status and quality of life.

9.
Clin Exp Rheumatol ; 40(1): 4-14, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-35088691

RESUMEN

Systemic lupus erythematosus (SLE) is a chronic multisystem auto-immune disease with extremely varied clinical manifestations and a complex pathogenesis. New insights in SLE about pathogenetic pathways, biomarkers, and data on clinical manifestations are progressively emerging, and new drugs and new therapeutic strategies have been proposed to improve the control of disease activity. Thus, this review is aimed to summarise the most relevant data about SLE emerged during 2021, following the previous annual review of this series.


Asunto(s)
Lupus Eritematoso Sistémico , Biomarcadores , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico
10.
Clin Exp Rheumatol ; 39(2): 231-241, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33843578

RESUMEN

In 2020 many contributions have been produced on SLE. Our critical digest of the recent literature will be focused on genetic factors that contribute to the development of the disease, novel potential therapeutic targets (including IL-23, IL-17, interferons and JAKs), diagnostic and prognostic biomarkers, classification criteria, clinical manifestations and comorbidities. We will then present new treatment options (with a special focus on belimumab, anifrolumab, tacrolimus, voclosporin and EULAR/ERA-EDTA recommendations for the management of LN) and treat-to-target strategy. Lastly, we will concentrate on some of the aspects that influence patients' disease perception and quality of life.


Asunto(s)
Lupus Eritematoso Sistémico , Calidad de Vida , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico
11.
Lupus ; 30(3): 439-447, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33413007

RESUMEN

OBJECTIVE: to describe the disease path and the very long-term outcome in a monocentric cohort of patients with Systemic Lupus Erythematosus (SLE). METHODS: SLE patients with a disease duration of at least 15 years from diagnosis were enrolled. The number of hospitalizations, the disease flares occurred over the disease course and the organ damage accumulation were evaluated at 1, 2, 3, 4, 5, 10 years from diagnosis and at last observation in 2019 as well. Disease state, ongoing therapies and quality of life measures were also assessed at last visit. RESULTS: 126 Caucasian SLE patients were included in the analysis (95% female, median age 47.5 IQR 41-53, median disease duration 21 IQR19-26). At last visit, the majority of the patients (78.6%) was on LLDAS (remission included), 53.4% were on GC treatment and 35.7% on immunosuppressant. Furthermore, 53.2% had at least one organ damage. The majority of patients (66.7%) presented a relapsing-remitting course, for a total of 158 flares during the disease course (incidence rate: 0.79/patient-year); moreover, 84.9% of the cohort experienced at least one hospital admission, amounting to a total of 328 hospitalizations (incidence rate: 0.85/patient-year). The main reason for admission was disease activity, while the percentage of hospitalizations due to other causes has been growing over the 10 years of follow-up. CONCLUSION: after a very long period of disease, most of the patients with SLE are in remission and are not taking GC therapy; however, the risk of incurring in disease flare remains a real problem.


Asunto(s)
Progresión de la Enfermedad , Lupus Eritematoso Sistémico/complicaciones , Calidad de Vida , Inducción de Remisión , Brote de los Síntomas , Adulto , Femenino , Glucocorticoides/uso terapéutico , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo
12.
PLoS One ; 16(1): e0245274, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33439910

RESUMEN

INTRODUCTION: The aim of this study was to investigate the incidence and clinical presentation of SARS-CoV-2 infections in a Systemic Lupus Erythematosus (SLE) cohort; to assess correlations with disease characteristics and rheumatic therapy; and to evaluate the occurrence of treatment discontinuation and its impact on disease activity. MATERIALS AND METHODS: SLE patients monitored by a single Italian centre were interviewed between February and July 2020. Patients were considered to be positive for SARS-CoV-2 infections in case of 1) positive nasopharyngeal swab; 2) positive serology associated with COVID19 suggesting symptoms. The following data were also recorded: clinical symptoms, adoption of social distancing measures, disease activity and treatment discontinuation. RESULTS: 332 patients were enrolled in the study. Six patients (1.8%) tested positive for SARS-CoV-2 infection, with the incidence being significantly higher in the subgroup of patients treated with biological Disease-Modifying Anti-Rheumatic Drugs (p = 0.005), while no difference was observed for other therapies, age at enrollment, disease duration, type of cumulative organ involvement or adoption of social isolation. The course of the disease was mild. Thirty-six patients (11.1%) discontinued at least part of their therapy during this time period, and 27 (8.1%) cases of disease flare were recorded. Correlation between flare and discontinuation of therapy was statistically significant (p<0.001). No significant increase of rate of flare in a subgroup of the same patients during 2020 was observed. CONCLUSION: Treatment discontinuation seems to be an important cause of disease flare. Our findings suggest that abrupt drug withdrawal should be avoided or evaluated with caution on the basis of individual infection risk and comorbidities.


Asunto(s)
COVID-19/complicaciones , COVID-19/epidemiología , Lupus Eritematoso Sistémico/complicaciones , Estudios de Cohortes , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Medición de Riesgo
13.
Arthritis Res Ther ; 22(1): 241, 2020 10 14.
Artículo en Inglés | MEDLINE | ID: mdl-33054823

RESUMEN

BACKGROUND: Remission or the lowest possible disease activity is the main target in the management of systemic lupus erythematosus (SLE). Anyway, conflicting data are present in the literature regarding the correlation between physician-driven definitions and patient perception of the disease. The objective of this study is to evaluate the relationship between the definition of lupus low disease activity state (LLDAS) and patient's health-related quality of life (HRQoL). METHODS: This is a cross-sectional, monocentric study. Adult SLE patients were included. For each patient, demographics, disease duration, medications, comorbidities, organ damage, active disease manifestations and SELENA-SLEDAI were assessed. Patients have been categorised as follows: LLDAS, remission and active disease. Each patient completed the following patient-reported outcomes (PROs): SF-36, LIT, FACIT-Fatigue and SLAQ. A SLAQ score < 6 (25° percentile of our cohort) was used as the cut-off value to define a low disease activity state according to patient self-evaluation. RESULTS: We enrolled 259 consecutive SLE patients (mainly female and Caucasian, mean age 45.33 ± 13.14 years, median disease duration 14 years). 80.3% were in LLDAS, of whom 82.2% were in remission; 19.7% were active. No differences emerged for any of the PROs used between the LLDAS and the active group. Considering the LLDAS subgroup, we identified 56 patients with a subjective low disease activity (SLAQ < 6) and we defined them as "concordant"; the remaining 152 patients in LLDAS presented a subjective active disease (SLAQ ≥ 6) and were defined "discordant". Discordant patients presented more frequently ongoing and past joint involvement (p < 0.05) and a diagnosis of fibromyalgia (p < 0.01); furthermore, they were more likely to be on glucocorticoid therapy (p < 0.01). Discordant patients showed a significantly poorer HRQoL, assessed by all PROs (p < 0.0001). CONCLUSIONS: Joint involvement, glucocorticoid therapy and comorbid fibromyalgia resulted to be the most important variables determining the poor concordance between patient and physician perspective on the disease.


Asunto(s)
Fibromialgia , Lupus Eritematoso Sistémico , Médicos , Adulto , Estudios Transversales , Femenino , Fibromialgia/diagnóstico , Fibromialgia/tratamiento farmacológico , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Persona de Mediana Edad , Calidad de Vida , Índice de Severidad de la Enfermedad , Esteroides
14.
Lupus ; 29(10): 1198-1205, 2020 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-32660311

RESUMEN

OBJECTIVES: The Brief Index of Lupus Damage (BILD) is an instrument of self-evaluation of organ damage for systemic lupus erythematosus (SLE) patients. The objectives of this study were the translation, cultural adaptation and validation of the Italian version of the BILD (BILDit). METHODS: The process of translation and cultural adaptation followed published guidelines. The BILDit was pretested in a pilot study with 30 SLE patients in order to evaluate acceptability, reliability, comprehension and feasibility, and then validated in consecutive SLE patients attending our clinic. RESULTS: A total of 167 SLE patients were enrolled. In the pilot study, the BILDit demonstrated good acceptability, feasibility and comprehensibility and a very high degree of reliability (Cronbach's α = 1). In the validation cohort, the BILDit showed a significant positive correlation with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI; ρ = 0.69; p < 0.001). Analysing the item-by-item correlation between the BILDit and the SDI, a good correlation (p < 0.001) was found for 73.1% of the items. In the multivariate analysis, the BILDit showed a significant positive correlation with age and disease duration (p < 0.01). CONCLUSIONS: The BILDit seems to be an acceptable and reliable instrument for patient self-evaluation of disease damage, with a good correlation with the SDI. It can be considered as a screening tool for the evaluation of organ damage starting from the patient's perceptive.


Asunto(s)
Lupus Eritematoso Sistémico/complicaciones , Encuestas y Cuestionarios/normas , Adulto , Comparación Transcultural , Progresión de la Enfermedad , Femenino , Humanos , Italia , Lupus Eritematoso Sistémico/fisiopatología , Masculino , Persona de Mediana Edad , Proyectos Piloto , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad , Traducciones
15.
Clin Exp Rheumatol ; 38(4): 592-601, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32662410

RESUMEN

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a relapsing-remitting course that can affect various organs or systems, leading to a broad spectrum of clinical manifestations. In the past year, many studies have been published on SLE, providing a significant advancement in disease knowledge and patient management. The aim of this review is to summarise the most relevant scientific contributions on SLE pathogenesis, clinical manifestations and comorbidities, biomarkers and treatment strategies published in 2019.


Asunto(s)
Lupus Eritematoso Sistémico , Biomarcadores , Comorbilidad , Humanos
17.
Ann Rheum Dis ; 79(7): 943-950, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32321721

RESUMEN

OBJECTIVES: Remission in systemic lupus erythematosus (SLE) is defined through a combination of 'clinical SLE Disease Activity Index (cSLEDAI)=0', 'physician's global assessment (PGA) <0.5' and 'prednisone (PDN) ≤5 mg/day'. We investigated the performance of these items, alone or in combination, in defining remission and in predicting SLICC/ACR Damage Index. METHODS: We tested seven potential definitions of remission in SLE patients followed-up for ≥5 years: PDN ≤5 mg/day; PGA <0.5; cSLEDAI=0; PGA <0.5 plus PDN ≤5 mg/day; cSLEDAI=0 plus PGA <0.5; cSLEDAI=0 plus PDN ≤5 mg/day; cSLEDAI=0 plus PDN ≤5 mg/day plus PGA <0.5. The effect of these definitions on damage was evaluated by Poisson regression analysis; the best performance was identified as the lowest Akaike and Bayesian information criterion (AIC and BIC). Positive and negative predictive values in identifying no damage increase were calculated. RESULTS: We included 646 patients (mean±SD disease duration 9.2±6.9 years). At multivariate analysis, ≥2 consecutive year remission according to all definitions protected against damage (OR, 95% CI: PGA <0.5 0.631, 0.444 to 0.896; cSLEDAI=0 0.531, 0.371 to 0.759; PGA <0.5 plus PDN ≤5 mg/day 0.554, 0.381 to 0.805; cSLEDAI=0 plus PGA <0.5 0.574, 0.400 to 0.826; cSLEDAI=0 plus PDN ≤5 mg/day 0.543, 0.376 to 0.785; cSLEDAI=0 plus PDN ≤5 mg/day plus PGA <0.5 0.532, 0.363 to 0.781, p<0.01 for all), except PDN ≤5 mg/day, which required four consecutive years (OR 0.534, 95% CI 0.325 to 0.877, p=0.013). Positive and negative predictive values were similar; however, cSLEDAI=0 showed the best performance (AIC 1082.90, BIC 1109.72, p<0.0001). Adding PGA <0.5 and/or PDN ≤5 mg/day to cSLEDAI=0 decreased remission duration (-1.8 and -1.5 year/patient, respectively) without increasing cSLEDAI=0 performance in predicting damage accrual. CONCLUSIONS: cSLEDAI=0 is the most attainable definition of remission, while displaying the best performance in predicting damage progression in the short-to-mid-term follow-up.


Asunto(s)
Lupus Eritematoso Sistémico/clasificación , Lupus Eritematoso Sistémico/tratamiento farmacológico , Evaluación de Resultado en la Atención de Salud/métodos , Índice de Severidad de la Enfermedad , Adulto , Antiinflamatorios/administración & dosificación , Teorema de Bayes , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Humanos , Italia , Masculino , Persona de Mediana Edad , Análisis Multivariante , Prednisona/administración & dosificación , Análisis de Regresión , Inducción de Remisión
18.
RMD Open ; 6(1)2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-32075876

RESUMEN

BACKGROUND: Fatigue is a very common and debilitating symptom in patients with systemic lupus erythematosus (SLE), even among those with a mild or inactive disease. The objective of this study is to define fatigue determinants and describe the impact of fatigue on health-related quality of life (HRQoL) and illness perception in a monocentric cohort of patients with SLE. METHODS: This is a cross-sectional study. Adult patients with SLE were included. For each patient, demographics, medications, comorbidities, organ damage (Systemic Lupus International Collaborating Clinics Damage Index), active disease manifestations and Systemic Lupus Disease Activity Index scores were collected. It was evaluated if each patient met the definitions of remission and low disease activity. At enrolment, each patient completed the Short Form-36 (SF-36), Functional Assessment Chronic Illness Therapy-Fatigue (FACIT-F), Lupus Impact Tracker (LIT), Systemic Lupus Activity Questionnaire (SLAQ) and Brief Index of Lupus Damage (BILD). The FACIT-F questionnaire was also administered to a group of healthy controls. RESULTS: 223 patients were included (mean age 44.9±13.2 years, median disease duration 13 years). 18.2% had an active disease, 43.5% met the definition of remission on treatment, and 11.8% had a concomitant fibromyalgia. The median FACIT-F score of our cohort was significantly lower compared with that of healthy controls (40 vs 47; p<0.001). FACIT-F scores were irrespective of age, disease duration, disease activity and damage. FACIT-F score was significantly lower in patients with fibromyalgia (p<0.01). FACIT-F scores demonstrated a significant correlation with all other patient-reported outcomes: SF-36 (r=0.53-0.77), LIT (r=-0.78), SLAQ (r=-0.72) and BILD (r=-0.28). CONCLUSIONS: Fatigue in patients with SLE has a strong negative impact on HRQoL and patient perception of the disease burden. Fatigue seems irrespective of disease activity but significantly influenced by the presence of fibromyalgia.


Asunto(s)
Fatiga/psicología , Fibromialgia/complicaciones , Lupus Eritematoso Sistémico/psicología , Calidad de Vida/psicología , Adulto , Estudios de Casos y Controles , Estudios de Cohortes , Costo de Enfermedad , Estudios Transversales , Fatiga/etiología , Femenino , Fibromialgia/epidemiología , Fibromialgia/psicología , Humanos , Lupus Eritematoso Sistémico/complicaciones , Masculino , Persona de Mediana Edad , Medición de Resultados Informados por el Paciente , Percepción , Índice de Severidad de la Enfermedad , Encuestas y Cuestionarios
19.
Clin Exp Rheumatol ; 37(5): 715-722, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31376267

RESUMEN

Systemic lupus erythematosus (SLE) is an autoimmune connective-tissue disorder with a wide range of clinical manifestations that predominantly affect women. Many aspects of its pathogenesis are still unclear, and new therapeutic strategies are progressively emerging. Thus, in this review we aim to summarise the most relevant data on SLE that emerged during 2018, following the previous annual review of this series. In particular, the review will focus on new insights in SLE regarding new pathogenetic pathways, new biomarkers, new data on clinical manifestations, clinical outcomes and comorbidities and what has emerged on new drugs and new therapeutic strategies.


Asunto(s)
Biomarcadores , Lupus Eritematoso Sistémico , Autoinmunidad , Biomarcadores/metabolismo , Comorbilidad , Femenino , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/terapia , Masculino , Pronóstico , Factores de Riesgo
20.
RMD Open ; 5(2): e000916, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31275608

RESUMEN

Objectives: To evaluate the proportion of patients who have successfully withdrawn glucocorticoids (GCs) in a longitudinal cohort of patients with systemic lupus erythematosus (SLE) over a period of 6 years; to evaluate patient characteristics during GC withdrawal in relation to existing definitions of remission and Lupus Low Disease Activity State (LLDAS); and to evaluate the occurrence of flares after GC withdrawal. Methods: Patients who attempted GC withdrawal were identified for the cohort, and the following information was assessed during withdrawal attempts: date of last disease flare, disease activity and damage and ongoing treatment. Information regarding the occurrence of disease flares after GC withdrawal was also recorded for patients who successfully stopped treatment.Definitions of remission were applied to GC withdrawal in line with European consensus criteria (Definitions of remission in SLE [DORIS]) and LLDAS in line with the Asian Pacific Lupus Consortium definition. Results: 148 patients were involved in the study; GC withdrawal was attempted in 91 patients (61.5%) with 77 patients (84.6%) successfully stopping GCs. At the beginning of the GC reduction, the majority of patients were in complete or clinical remission (48.9% and 39.6%, respectively). Disease activity was significantly lower in patients who successfully stopped GCs, and the proportion of patients in complete remission was higher (54.2%) with respect to patients who failed in their attempt. Among patients who stopped GCs, 18 flares were recorded after a median of 1 year. The time period since the last flare was shorter in patients who experienced flares with respect to patients who did not flare (mean 0.93 years vs 6.0, p<0.001). Conclusions: GC withdrawal is an achievable goal in SLE and may be attempted after a long-term remission or LLDAS to protect the patient from disease flares.


Asunto(s)
Glucocorticoides/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Privación de Tratamiento/estadística & datos numéricos , Adulto , Manejo de la Enfermedad , Progresión de la Enfermedad , Humanos , Estudios Longitudinales , Persona de Mediana Edad , Inducción de Remisión/métodos , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
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