Publications by authors named "Elena Elefante"

41 Publications

One year in review 2021: systemic lupus erythematosus.

Clin Exp Rheumatol 2021 Mar-Apr;39(2):231-241. Epub 2021 Apr 9.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
April 2021

Are remission and low disease activity state ideal targets for pregnancy planning in Systemic Lupus Erythematosus? A multicentre study.

Rheumatology (Oxford) 2021 Feb 16. Epub 2021 Feb 16.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keab155DOI Listing
February 2021

COVID-19: the new challenge for rheumatologists. One year later.

Clin Exp Rheumatol 2021 Jan-Feb;39(1):203-213. Epub 2021 Feb 5.

University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
February 2021

Impact of first wave of SARS-CoV-2 infection in patients with Systemic Lupus Erythematosus: Weighting the risk of infection and flare.

PLoS One 2021 13;16(1):e0245274. Epub 2021 Jan 13.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0245274PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7806138PMC
January 2021

Myeloid neoplasms and autoimmune diseases: markers of association.

Clin Exp Rheumatol 2021 Jan 6. Epub 2021 Jan 6.

Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
January 2021

How do systemic lupus erythematosus patients with very-long disease duration present? Analysis of a monocentric cohort.

Lupus 2021 Mar 7;30(3):439-447. Epub 2021 Jan 7.

Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0961203320984230DOI Listing
March 2021

The patient's perspective: are quality of life and disease burden a possible treatment target in systemic lupus erythematosus?

Rheumatology (Oxford) 2020 12;59(Suppl5):v63-v68

Department Rheumatology & Hiller-Research Unit Rheumatology, Heinrich-Heine-University Düsseldorf, Medical Faculty, Düsseldorf, Germany.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keaa427DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7719037PMC
December 2020

Articular involvement, steroid treatment and fibromyalgia are the main determinants of patient-physician discordance in systemic lupus erythematosus.

Arthritis Res Ther 2020 10 14;22(1):241. Epub 2020 Oct 14.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02334-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7559765PMC
October 2020

One year in review 2020: systemic lupus erythematosus.

Clin Exp Rheumatol 2020 Jul-Aug;38(4):592-601. Epub 2020 Jul 10.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
September 2020

Translation, cultural adaptation and validation of the Italian version of the Brief Index of Lupus Damage: the BILDit.

Lupus 2020 Sep 13;29(10):1198-1205. Epub 2020 Jul 13.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

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;  < 0.001). Analysing the item-by-item correlation between the BILDit and the SDI, a good correlation ( < 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 ( < 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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0961203320940012DOI Listing
September 2020

Multicentric study comparing cyclosporine, mycophenolate mofetil and azathioprine in the maintenance therapy of lupus nephritis: 8 years follow up.

J Nephrol 2021 04 27;34(2):389-398. Epub 2020 May 27.

Divisione di Nefrologia e Dialisi-Padiglione Croff, Fondazione Ca' Granda IRCCS Ospedale Maggiore Policlinico Milano, Via della Commenda 15, 20122, Milano, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s40620-020-00753-wDOI Listing
April 2021

Are patients with systemic lupus erythematosus more prone to result false-positive for SARS-CoV2 serology?

Clin Exp Rheumatol 2020 May-Jun;38(3):577. Epub 2020 May 27.

Rheumatology Unit, University of Pisa, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
September 2020

One year in review 2020: vasculitis.

Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):3-14. Epub 2020 Apr 28.

Clinic of Rheumatology, Department of Medicine, University of Udine, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
September 2020

COVID-19: the new challenge for rheumatologists.

Clin Exp Rheumatol 2020 Mar-Apr;38(2):175-180. Epub 2020 Mar 24.

University of Pisa, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
March 2020

Impact of fatigue on health-related quality of life and illness perception in a monocentric cohort of patients with systemic lupus erythematosus.

RMD Open 2020 02;6(1)

Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/rmdopen-2019-001133DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7046978PMC
February 2020

Framingham, ACC/AHA or QRISK3: which is the best in systemic lupus erythematosus cardiovascular risk estimation?

Clin Exp Rheumatol 2020 Jul-Aug;38(4):602-608. Epub 2019 Oct 28.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
September 2020

Sexual dysfunction in Behçet's syndrome.

Rheumatol Int 2020 Jan 8;40(1):9-15. Epub 2019 Oct 8.

Rheumatology Unit, University of Pisa, Via Roma 67, 56126, Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00296-019-04455-wDOI Listing
January 2020

Pregnancy and undifferentiated connective tissue disease: outcome and risk of flare in 100 pregnancies.

Rheumatology (Oxford) 2020 06;59(6):1335-1339

Rheumatology Unit, Department of Clinical and Experimental Medicine, Pisa.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/kez440DOI Listing
June 2020

One year in review 2019: systemic lupus erythematosus.

Clin Exp Rheumatol 2019 Sep-Oct;37(5):715-722. Epub 2019 Jul 19.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
October 2019

Glucocorticoid withdrawal in systemic lupus erythematosus: are remission and low disease activity reliable starting points for stopping treatment? A real-life experience.

RMD Open 2019 11;5(2):e000916. Epub 2019 Jun 11.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/rmdopen-2019-000916DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6579574PMC
April 2020

One year in review 2019: vasculitis.

Clin Exp Rheumatol 2019 Mar-Apr;37 Suppl 117(2):3-19. Epub 2019 May 21.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Systemic vasculitis are disabling complex disorders potentially involving any organ and system. Tremendous efforts have been made recently in this field with novel insights into pathogenesis and new therapy in the pipeline. Following the previous annual reviews of this one year in review series, in this paper we provide a critical digest of the most recent literature regarding pathogenesis, clinical manifestations and therapy, with the ultimate aim of addressing whether the existing data may open new avenues for precision medicine in these disorders.
View Article and Find Full Text PDF

Download full-text PDF

Source
June 2019

First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry).

Front Cardiovasc Med 2019 28;6:11. Epub 2019 Feb 28.

Unità di Reumatologia, Dipartimento di Medicina, Università di Padova, Padova, Italy.

Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge. Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) or neonatal period (up to 27 days after birth). Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 , five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion ( = 0.005, OR > 100; CI 95% 2.88->100 and hydrops ( = 0.003, = 14.09; CI 95% 2.01-122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins. This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fcvm.2019.00011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6404544PMC
February 2019

Behçet's syndrome and psychiatric involvement: is it a primary or secondary feature of the disease?

Clin Exp Rheumatol 2018 Nov-Dec;36(6 Suppl 115):125-128. Epub 2018 Dec 13.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Objectives: The primary aim of the study was to determine the frequency of psychiatric disorders in Behçet's syndrome (BS) patients, both with and without neurological involvement. The secondary aims were: to investigate a possible association between disease activity/organ involvement/demographic data and psychiatric profile in BS patients, and to compare the distribution of psychiatric disorders in BS patients compared to patients with other chronic diseases.

Methods: One hundred and sixteen BS patients were studied; in addition, two groups of patients affected by systemic lupus erythematosus and chronic arterial hypertension were included in the study as disease control groups. The end-point was represented by the assessment of disease activity, performed by the evaluation of: the presence/absence of manifestations, BDCAF and clinician's overall perception of disease activity. Psychiatric comorbidity was evaluated according to the DSMIV-TR criteria.

Results: The frequency of bipolar disorders resulted significantly higher in BS than in disease controls. The presence of bipolar disorders in BS patients does not seem to be related to the presence of neurological involvement in the history of the disease. Notably, a significant correlation was found between BS disease activity and mood disorders, also in the follow-up.

Conclusions: The study demonstrated a high frequency of psychiatric disorders in BS patients, peculiarly represented by bipolar disorders. The presence of this involvement, independently from the organ involvement, and strictly related to the disease activity, seems to suggest that neuro-psycho-BS may represent an intrinsic aspect of BS.
View Article and Find Full Text PDF

Download full-text PDF

Source
March 2019

Disease activity assessment of rheumatic diseases during pregnancy: a comprehensive review of indices used in clinical studies.

Autoimmun Rev 2019 Feb 18;18(2):164-176. Epub 2018 Dec 18.

Department of Medicina dei Sistemi, Rheumatology, Allergology and Clinical Immunology, University of Rome Tor Vergata, Rome, Italy.

Pregnancy requires a special management in women with inflammatory rheumatic diseases (RDs), with the aim of controlling maternal disease activity and avoiding fetal complications. Despite the heterogeneous course of RDs during pregnancy, their impact on pregnancy largely relates to the extent of active inflammation at the time of conception. Therefore, accurate evaluation of disease activity is crucial for the best management of pregnant patients. Nevertheless, there are limitations in using conventional measures of disease activity in pregnancy, as some items included in these instruments can be biased by symptoms or by physiological changes related to pregnancy and the pregnancy itself may influence laboratory parameters used to assess disease activity. This article aims to summarize the current literature about the available instruments to measure disease activity during pregnancy in RDs. Systemic lupus erythematosus is the only disease with instruments that have been modified to account for several adaptations which might interfere with the attribution of signs or symptoms to disease activity during pregnancy. No modified-pregnancy indices exist for women affected by other RDs, but standard indices have been applied to pregnant patients. The current body of knowledge shows that the physiologic changes that occur during pregnancy need to be either adapted from existing instruments or developed to improve the management of pregnant women with RDs. Standardized instruments to assess disease activity during pregnancy would be helpful not only for clinical practice but also for research purposes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.autrev.2018.08.008DOI Listing
February 2019

Tacrolimus in non-Asian patients with SLE: a real-life experience from three European centres.

Lupus Sci Med 2018 2;5(1):e000274. Epub 2018 Nov 2.

Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy.

Objectives: To analyse the real-life practice on the use of Tacrolimus (TAC) in patients with systemic lupus erythematosus (SLE) from three European SLE referral centres.

Methods: Adult patients with SLE regularly followed at three European referral centres were included. Demographics, cumulative organ involvement, treatment history, Systemic Lupus Disease Activity Index (SLEDAI), laboratory features and physician's judgement were collected at baseline and at 3-6-12 months after starting TAC.

Results: 29 patients were included (89% female, mean age 38±9 years). Ethnicity was predominantly Caucasian (82%), Black African (11%), Hispanic (3.5%) and Caribbean (3.5%). The main indications for TAC prescription were renal involvement (82.7%), arthritis (10.3%), cutaneous manifestations (6.8%), haematological manifestations (6.8%), serositis (3.4%). At 3 months, there was a clinical improvement in 21 patients (72.4%) and 9 of these experienced a complete resolution of symptoms (31%). This corresponds to: (1) a significant decrease in the mean SLEDAI; (2) a significant decrease in the mean 24  hours proteinuria; a significant increase in C3 and stable creatinine values. At 6 months (n=25), the physician declared an improvement in 19 patients (76%) and a complete resolution of symptoms in 9 (36%). The same trend was observed at 12 months of follow-up. TAC was discontinued in nine pts (31%); reasons for discontinuation were inefficacy (13.8%), drug intolerance (10%) and disease remission (6.9%).

Conclusions: Despite the limitation due to the small number of patients and the uncontrolled nature of the study, these data show that TAC can be considered a valid therapeutic option in patients with SLE, especially for renal involvement.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/lupus-2018-000274DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257376PMC
November 2018

Imaging of joints in systemic lupus erythematosus.

Clin Exp Rheumatol 2018 Sep-Oct;36 Suppl 114(5):68-73. Epub 2018 Oct 1.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Musculoskeletal symptoms are among the most common manifestations in patients with systemic lupus erythematosus (SLE), being reported in up to 95% of patients; joint and tendon involvement can range from arthralgia to severe deforming arthropathy; while myositis a rare manifestation, comorbid fibromyalgia is reported in up to 40% of SLE patients. All these manifestations have a significant impact on the patients' quality of life, possibly leading to disability and functional impairment in daily living activities. In recent years, thanks to the availability of new imaging techniques for the assessment of tendon and joint pathologies, the approach to the definition and characterisation of these manifestations in SLE is constantly evolving. In this review we will therefore illustrate the state of the art of imaging techniques in the assessment of joint involvement in SLE, focusing on ultrasounds (US) and magnetic resonance (MRI), discussing their advantages, drawbacks and possible future developments. The main findings that emerge from the recent literature is that imaging studies may allow a more accurate definition of disease subtypes revealing an unexpected higher prevalence of joint and tendon involvement with respect to what known by clinical evaluation and standard radiography. Indeed, US and MRI also made possible the identification of joints and tendons pathologies in patients with no or very mild clinical symptoms. On the other hand, the interpretation of some findings remains uncertain, as well as the validity and feasibility of this analysis in clinical practice. Thus, further studies should clarify the clinical meaning of subclinical abnormalities detected in US and MRI scans and their impact on the long-term outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
January 2019

One year in review 2018: systemic lupus erythematosus.

Clin Exp Rheumatol 2018 Sep-Oct;36(5):763-777. Epub 2018 Sep 26.

Rheumatology Unit, Department of Medicine, University of Perugia, Italy.

Systemic lupus erythematosus (SLE) is a systemic autoimmune condition characterised by a wide spectrum of clinical manifestations, partly related to the disease itself, but also linked to its comorbidities and drugs adverse reactions. Following the previous annual reviews, we focused on new insights in SLE clinical features, pathogenic pathways, biomarkers of specific organ involvement and therapeutic strategies. We finally concentrated on SLE aspects that could significantly influence patients' quality of life and that need to be investigated in detail through the development and validation of disease-specific patient-reported outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
January 2019

One year in review 2018: systemic vasculitis.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):12-32. Epub 2018 May 18.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Systemic vasculitis are heterogeneous, complex and disabling disorders. Following the previous annual reviews of this series, this paper gives a brief overview on current knowledge about recent literature on small- and large-vessel systemic vasculitis, with a specific focus on pathogenetic and clinical aspects, novel possible disease-related biomarkers and current and future therapies that are in the pipeline.
View Article and Find Full Text PDF

Download full-text PDF

Source
July 2018