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1.
Clin Exp Rheumatol ; 39(2): 231-241, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33843578

RESUMO

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.

2.
Clin Exp Rheumatol ; 39(1): 203-213, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33555253

RESUMO

At the beginning of COVID-19, we underlined that this pandemic was a new challenge for rheumatologists. On the one hand, it was necessary to clarify the impact of this new viral disease on the natural history of many rheumatic diseases and, on the other hand, to define the beneficial or harmful effects of the synthetic or targeted therapies used for their treatment. In addition, we have postulated that in view of the common pathogenetic mechanisms involved, the therapeutic armamentarium currently employed in the management of viral or idiopathic systemic autoimmune rheumatic diseases could be useful to control the "cytokine storm" induced by SARS-COV-2. One year later, in the present review we have analysed the progress of the knowledge on both these aspects and updated the algorithms initially proposed for a rational use of the synthetic and targeted anti-inflammatory and immunomodulatory agents in the management of COVID-19.


Assuntos
Reumatologistas , Síndrome da Liberação de Citocina , Humanos , Pandemias
3.
Artigo em Inglês | MEDLINE | ID: mdl-33590843

RESUMO

OBJECTIVES: To determine whether disease remission or low disease activity state at the beginning of pregnancy in SLE patients is associated with better pregnancy outcome. METHODS: pregnancies in SLE patients prospectively monitored by pregnancy clinics at four rheumatology centres were enrolled. Patient demographics and clinical information were collected at baseline (pregnancy visit before 8 weeks of gestation) including whether patients were in remission according to DORIS criteria and and/or Lupus Low Disease Activity State (LLDAS). Univariate and multivariate analysis were performed to determine predictors of disease flare and adverse pregnancy outcomes (APOs) including preeclampsia, preterm delivery, small for gestational age infant, intrauterine growth restriction and intrauterine fetal death. RESULTS: 347 pregnancies were observed in 281 SLE patients. Excluding early pregnancy losses, 212 pregnancies (69.7%) occurred in patients who were in remission at baseline, 33 (10.9%) in patients in LLDAS, and the remainder in active patients. 73 flares (24%) were observed during pregnancy or puerperium, and 105 (34.5%) APOs occurred. Multivariate analysis revealed that patients in disease remission or taking hydroxychloroquine were less likely to have disease flare, while a history of lupus nephritis increased the risk. The risk of APOs was increased in patients with shorter disease duration, while being on hydroxychloroquine resulted a protective variable. An almost significant association between complete remission and a decreased risk of APOs was observed. CONCLUSIONS: Prenatal planning with a firm treat-to-target goal of disease remission is an important strategy to reduce the risk of disease flares and severe obstetrical complications in SLE pregnancies.

4.
Clin Exp Rheumatol ; 2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33427624

RESUMO

OBJECTIVES: To investigate the prognostic significance of concomitant autoimmune diseases (ADs) in myeloproliferative neoplasms (MPNs). METHODS: 435 subjects with a diagnosis of MPNs were included in this observational single institution longitudinal study. Of them, 34 patients presented an overt AD at diagnosis of MPN. Clinical presenting features, progression-free and overall survival were compared between MPN subgroups in relation to co-existence of AD at diagnosis of MPN. RESULTS: Compared to cases without ADs, the subjects with ADs were significantly younger, had lower haemoglobin and haematocrit levels and more frequently presented with splenomegaly. The clinical and biological features associated to progression-free and overall survival were: age, presence of splenomegaly, histotype (MF vs. PV vs. ET), anaemia, high platelet count and presence of any AD at diagnosis of MPN. The age-adjusted hazard ratio (HR) of progression for the presence of AD at diagnosis of MPN was 2.76. Overall survival was not significantly associated to AD at diagnosis, but the HR of progression for the presence of AD at diagnosis of MPN was 2.18. CONCLUSIONS: A possible common genetic predisposition, the inflammatory bone marrow microenvironment and the activation of theJAK/STAT pathway could be considered as responsible for the observed association between MPNs and ADs.

5.
PLoS One ; 16(1): e0245274, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33439910

RESUMO

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.


Assuntos
/complicações , Lúpus Eritematoso Sistêmico/complicações , Estudos de Coortes , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Medição de Risco
6.
Lupus ; 30(3): 439-447, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33413007

RESUMO

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.

7.
Rheumatology (Oxford) ; 59(Supplement_5): v63-v68, 2020 Dec 05.
Artigo em Inglês | MEDLINE | ID: mdl-33280017

RESUMO

A few decades ago, the therapy goal of patients with systemic lupus erythematosus (SLE) was survival and the prevention of organ failure. Today, clinical remission and low disease activity are believed to be the optimal therapeutic targets. These aims are difficult to reach for many patients, but they still do not address the health-related quality of life (QoL) that is significantly impaired in SLE patients. Even in the state of remission, QoL and fatigue are insufficient controlled. Thus, patient-oriented research is essential to design new strategies for the management of lupus patients. The INTEGRATE project analyses the patients' and physicians' perspectives to pave the way to design an innovative therapeutic strategy for lupus and focuses on the multifaceted dimensions of the disease burden. Shared decision making (SDM) could include the patient's perspective of SLE to treatment strategy and consider QoL and the burden of lupus into the process of therapy decision.

8.
Arthritis Res Ther ; 22(1): 241, 2020 10 14.
Artigo em Inglês | MEDLINE | ID: mdl-33054823

RESUMO

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.

9.
Lupus ; 29(10): 1198-1205, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32660311

RESUMO

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.

10.
Clin Exp Rheumatol ; 38(4): 592-601, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32662410

RESUMO

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.


Assuntos
Lúpus Eritematoso Sistêmico , Biomarcadores , Comorbidade , Humanos
13.
Clin Exp Rheumatol ; 38 Suppl 124(2): 3-14, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32359039

RESUMO

Systemic vasculitides are a group of diseases that could potentially affect any organ with heterogeneous clinical manifestations that usually depend on the size of the most involved vessels. These diseases could be associated with a relevant burden of mortality and morbidity if not early recognised and treated. Moreover, even if they are usually rare diseases, their incidence and prevalence seem to be increasing in the last decade, partially because of improved awareness and management of vasculitis from physicians. Like in the previous annual reviews of this series, in this paper we revised the most recent literature on pathogenesis, clinical manifestations and treatment options in small- and large-vessel vasculitis.


Assuntos
Vasculite Sistêmica/diagnóstico , Vasculite Sistêmica/terapia , Humanos , Incidência , Prevalência
14.
J Nephrol ; 2020 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-32462476

RESUMO

BACKGROUND: The ideal long-term maintenance therapy of Lupus Nephritis (LN) is still a matter of debate. The present study was aimed at comparing the efficacy/safety profile of cyclosporine (CsA), mycophenolate mofetil (MMF) and azathioprine (AZA) in long-term maintenance therapy of LN. METHODS: We performed a retrospective study of patients with biopsy-proven active LN. After induction therapy, all patients received maintenance therapy with CsA, MMF or AZA based on medical decision. Primary endpoint was complete renal remission (CRR) after 8 years (defined as proteinuria < 0.5 g/24 h, eGFR > 60 ml/min/1.73 mq); secondary endpoints were: CRR after 1 year, renal and extrarenal flares, progression of chronic kidney disease (CKD stage 3 or above) and side-effects. RESULTS: Out of 106 patients, 34 received CsA, 36 MMF and 36 AZA. Clinical and histological characteristics at start of induction therapy were comparable among groups. At start of maintenance therapy, CsA patients had significantly higher proteinuria (P = 0.004) or nephrotic syndrome (P = 0.024) and significantly lower CRR (23.5% vs 55.5% on MMF and 41.7% on AZA, P = 0.024). At one year, CRR was similar in the three groups (79.4% on CsA, 63.8% on MMF, 58.3% on AZA, P = 0.2). At 8 years, the primary endpoint was achieved by 79.4% of CsA vs 83.3% of MMF and 77.8% of AZA patients (P = 0.83); 24 h proteinuria, serum creatinine, eGFR were similar. CKD stage 3 or above developed in 8.8% of CsA, in 8.3% of MMF and in 8.3% of AZA patients (P = 0.92). Flares-free survival curves and incidence of side-effects were not different. CONCLUSIONS: This is the first study comparing CsA, MMF and AZA on long-term LN maintenance therapy. All treatments had similar efficacy in achieving and maintaining CRR, despite more severe baseline clinical features in patients treated with CsA.

17.
RMD Open ; 6(1)2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-32075876

RESUMO

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.

18.
Rheumatology (Oxford) ; 59(6): 1335-1339, 2020 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-31593595

RESUMO

OBJECTIVE: UCTD is a systemic autoimmune condition that fails to fulfil the criteria for a definite CTD. Given that there are a lack of studies on links between pregnancy and UCTD, the purpose of this study was to evaluate the risk of disease flares or development of CTD in addition to the risk of adverse pregnancy outcomes in patients with UCTD. METHODS: This is a retrospective study using prospectively collected data for 100 pregnancies in 81 incidences of UCTD treated in a single referral centre. RESULTS: A total of 11 pregnancies (11%) ended in miscarriage in the first trimester and the remaining 89 (89%) ended with a live birth. Thirteen patients (13%) flared during pregnancy or puerperium and three (3%) suffered major flares that led to the development of SLE with renal involvement. Obstetric complications occurred in 26 of the 89 successful pregnancies (29%), including 1 case (1%) of pre-eclampsia; in some cases, a single pregnancy was affected by more than one complication. There was a significant link between disease flare and both anti-dsDNA-positive antibodies at baseline (P < 0.01) and disease activity at the beginning of pregnancy (P < 0.01). CONCLUSION: The impact on pregnancy in the study's cohort appears to be less serious in UCTD than in other CTDs. Nevertheless, disease flares and obstetric complications can represent a clinical challenge and clinical and serological disease activity would appear to represent important determinants of pregnancy outcomes. Pre-pregnancy counselling and planning as well as close monitoring during pregnancy is therefore essential.


Assuntos
Complicações na Gravidez/diagnóstico , Resultado da Gravidez , Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico , Adulto , Progressão da Doença , Feminino , Humanos , Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto Jovem
19.
Clin Exp Rheumatol ; 38(4): 602-608, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31694741

RESUMO

OBJECTIVES: Our objective was to compare three algorithms for cardiovascular (CV) risk estimation, namely Framingham, ACC/AHA and QRISK3, in a cohort of patients with systemic lupus erythematosus (SLE). METHODS: Consecutive patients with SLE according to the ACR criteria were enrolled. Traditional risk factors, ongoing therapies, comorbidities and SLE-specific evaluations were assessed. In those without previous myocardial infarction or stroke, Framingham, ACC/AHA and QRISK3 algorithms were then used to estimate the individual risk of developing a CV disease over the next 10 years. RESULTS: Patients eligible for CV risk estimation were 123 out of 135 enrolled. Framingham index reported a median risk score of 4.7% (IQR 9.5-2.2), considering 29 patients (23.6%) at high CV risk. ACC/AHA index showed a median risk score of 1.4% (IQR 4.5-0.7), with 17 patients (13.8%) at high-risk. QRISK3 revealed a median risk score of 6.2% (IQR 12.5-2.8), making it possible to classify 44 patients (35.8%) at high CV risk. The subgroup analysis of subjects older than 40 years confirmed the same number of high-risk patients for both Framingham and ACC/AHA, whereas QRISK3 classified 38 subjects at high CV risk. CONCLUSIONS: QRISK3 classifies a greater number of SLE patients at high-risk of developing CV diseases over the next 10 years in comparison with classic algorithms as Framingham and ACC/AHA. If its predictive accuracy were confirmed by longitudinal data, QRISK3 could become an important tool in the early detection of a considerable part of CV high-risk SLE patients that would be underestimated when applying classic algorithms.


Assuntos
Doenças Cardiovasculares , Lúpus Eritematoso Sistêmico , Infarto do Miocárdio , Humanos , Medição de Risco , Fatores de Risco
20.
Rheumatol Int ; 40(1): 9-15, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31595309

RESUMO

Behçet's syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis; articular, vascular, gastroenteric and neurological involvement may also occur. The multi-organ involvement and the wide range of clinical spectrum make the diagnosis of BS challenging. As other systemic chronic diseases, BS can affect all aspects of patients' life, including sexual dysfunction (SD). So far, SD has been deeply studied among patients affected by many rheumatic diseases; however, little is known about the prevalence and the characteristics of SD among BS patients. The present work was aimed at providing a systematic literature review of the literature published on SD and BS. We carried out a systematic search in PubMed and Scopus based on controlled terms (MeSH) and keywords to identify literature data on SD in BS. The systematic search was independently performed by two clinicians based on the controlled medical subject heading terms for Behcet syndrome and SD. Systematic database search identified 32 records. Screening by title and abstract was then conducted and a total of 10 articles were eligible for full text assessment, five studies explored SD in male patients with BS, 3 studies in females and 2 works reported data on both genders. Globally, the systematic literature review results have underlined that SD seems frequent in BS patients. Both female and male patients experienced a significant correlation between SD and depression. The studies investigating sexuality in BS seem to demonstrate that in these patients SD may be mainly related to depression rather than to active organic manifestations.

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