Publications by authors named "Viola Signorini"

15 Publications

  • Page 1 of 1

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.
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April 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.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0245274PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7806138PMC
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.
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http://dx.doi.org/10.1177/0961203320984230DOI Listing
March 2021

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

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.

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September 2020

Remission in systemic lupus erythematosus: testing different definitions in a large multicentre cohort.

Ann Rheum Dis 2020 07 22;79(7):943-950. Epub 2020 Apr 22.

Department of Medicine, Division of Rheumatology, University of Padua, Padova, Italy

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.
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http://dx.doi.org/10.1136/annrheumdis-2020-217070DOI Listing
July 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.
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http://dx.doi.org/10.1136/rmdopen-2019-001133DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7046978PMC
February 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.
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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.
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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 2018: progress in osteoporosis treatment.

Clin Exp Rheumatol 2018 Nov-Dec;36(6):948-958. Epub 2018 Dec 6.

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

Osteoporosis is a generalised bone disease characterised by decreased bone mass and deterioration of bone microarchitecture predisposing to fragility fractures. Bone fractures are a remarkable social and economic health problem, and several studies have been carried out in order to reduce their occurrence. Inhibiting bone resorption and increasing bone formation are the mainstay of treatment, anti-catabolic and anabolic, respectively. This review highlights the most recent advances in osteoporosis and reports the evidence of efficacy and safety of anabolic treatment of osteoporosis, as evaluated by randomised, controlled trials published during 2017. As the most common form of secondary osteoporosis, we will also discuss the 2017 state-of-the-art on pathogenesis and treatment of glucocorticoid-induced osteoporosis.
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March 2019

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.
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January 2019

One year in review 2016: systemic lupus erythematosus.

Clin Exp Rheumatol 2016 Jul-Aug;34(4):569-74. Epub 2016 Jul 14.

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

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a highly variable course and prognosis. The management of the disease is still a clinical challenge for the treating physicians as many aspects regarding the disease pathogenesis, clinical picture and outcomes remain to be elucidated. New and interesting data are emerging; here the recent literature on SLE pathogenesis, clinical and laboratory aspects, as well as treatments and comorbidities, are reviewed and the main findings summarised in order to provide a bird's eye on the relevant papers on these topics.
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September 2016

Leukopenia, lymphopenia, and neutropenia in systemic lupus erythematosus: Prevalence and clinical impact--A systematic literature review.

Semin Arthritis Rheum 2015 Oct 21;45(2):190-4. Epub 2015 May 21.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, Pisa 56100, Italy. Electronic address:

Objective: To systematically review the available evidence to evaluate (1) the prevalence and degree of leukopenia, lymphopenia, and neutropenia in patients with systemic lupus erythematosus (SLE), (2) whether these conditions carry a major infection risk for patients, and (3) whether a treatment with colony stimulating factors (CSF) can be an effective and safe option in SLE patients with leukopenia.

Material And Methods: MedLine and Embase were searched by including MeSH terms, text words, and subheadings "systemic lupus erythematosus," "leukopenia" (first search), and "colony stimulating factor" (second search). Inclusion and exclusion criteria were a priori defined and two reviewers screened the retrieved articles for selection criteria; data from the included studies were recorded in ad hoc standard forms; the results were synthesized and transported to evidence tables.

Results: A total of 17 articles were included in the systematic literature review: nine articles were retrieved for the first research question and 11 for the second while no articles satisfied the inclusion criteria for the third research question. The prevalence of leukopenia is reported in 22-41.8% of cases and lymphopenia is reported cumulatively from 15% to 82% of the patients while neutropenia is described in 20-40% of the patients. There is no evidence of a significant association between overall reduction of white blood cells and infection occurrence while some studies found a strong association between low lymphocytes/neutrophils count and the risk of major infections. Only case reports and case series have been found to investigate the safety of CSF in SLE patients.

Conclusions: The results of this systematic literature review are inconclusive for many aspects related to the original research questions and highlight the need for further studies. Indeed, the strength of the evidence is not sufficiently robust to draw specific recommendations on how to balance between the need to treat the patient with SLE with immunosuppressive drugs and the risk of severe infections.
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http://dx.doi.org/10.1016/j.semarthrit.2015.05.009DOI Listing
October 2015