22 results on '"Bellando-Randone S"'
Search Results
2. POS0044 DECIPHERING THE GUT MICROBIOTA OF VERY EARLY SYSTEMIC SCLEROSIS (VEDOSS) PATIENTS: A TAXONOMIC AND FUNCTIONAL CHARACTERIZATION
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Russo, E., primary, Lepri, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Ghezzi, G., additional, Gloria, L. DI, additional, Orlandi, M., additional, Bertorello, S., additional, Bruni, C., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional
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- 2024
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3. POS0425 SUBCLINICAL LOSS OF LUNG VOLUMES IN VERY EARLY SSc: EVIDENCE FROM THE VEDOSS DATABASE
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Kakkar, V., primary, Donato, S. DI, additional, Ross, R., additional, Wasson, C. W., additional, Bellando-Randone, S., additional, Matucci-Cerinic, M., additional, Allanore, Y., additional, and Del Galdo, F., additional
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- 2024
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4. POS1483-HPR THE TELEMEDICINE HETEROGENEITY IN RHEUMATIC AND MUSCULOSKELETAL DISEASES: A SCOPING REVIEW
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El Aoufy, K., primary, Melis, M. R., additional, Tamburini, M., additional, Moggi Pignone, A., additional, Bandini, G., additional, Bellando-Randone, S., additional, Matucci-Cerinic, M., additional, Rasero, L., additional, and Bambi, S., additional
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- 2024
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5. Rheumatic diseases in migrant patients resident in Tuscany: epidemiological data analysis and single-center experience.
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Cometi, L., Francesconi, P., Bellini, B., Turco, L., Voller, F., Nacci, F., Bellando-Randone, S., Matucci-Cerinic, M., and Guiducci, S.
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- 2024
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6. The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies.
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Scaletti C, Pratesi S, Bellando Randone S, Di Pietro L, Campochiaro C, Annunziato F, and Matucci Cerinic M
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Systemic sclerosis is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting in the production of autoantibodies. Abnormalities of the immune system compromise the normal function of blood vessels leading to a vasculopathy manifested by Raynaud's phenomenon, an early sign of systemic sclerosis. As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis. Several systemic sclerosis-specific autoantibodies are currently known and are associated with specific clinical manifestations and prognosis. Although the pathogenetic role of these autoantibodies is still unclear, their production by B cells and plasma cells suggests the importance of these cells in the development of systemic sclerosis. This review narratively examines B cell dysfunctions and their role in the pathogenesis of systemic sclerosis and discusses B cell-targeted therapies currently used or potentially useful for the management of end-organ complications., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology.)
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- 2024
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7. Doppler ultrasound, a noninvasive tool for the study of mesenteric arterial flow in systemic sclerosis: a cross-sectional study of a patient cohort with review and meta-analysis of the literature.
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Bandini G, Monami M, Ciuti G, Mercatelli P, Lo Cricchio A, De Santis MC, Bonomi F, Bellando Randone S, Campochiaro C, El Aoufy K, Ruaro B, Giuggioli D, Hughes M, McMahan ZH, Benfaremo D, Moroncini G, Maconi G, Accogli E, Dagna L, Matucci Cerinic M, and Moggi Pignone A
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Gastrointestinal involvement (GI) is a frequent and troublesome complication of systemic sclerosis (SSc), whose etiology is poorly understood, though it is hypothesized that autoimmunity and progressive vasculopathy may play a role. Vasculopathy is considered one of the main pathogenetic pathways responsible for many of the clinical manifestations of SSc, and, therefore, studying the principal splanchnic vessels (i.e., superior mesenteric artery-SMA and inferior mesenteric artery-IMA) with Doppler Ultrasound (DUS) may provide further insights into measuring the progression of vasculopathy, evaluating its possible association with SSc GI symptoms, and determining whether it plays a role in the development or severity of SSc GI disease. A cohort of SSc patients consecutively recruited underwent DUS examination, and associations with GI (UCLA-GIT 2.0 questionnaire) and extraintestinal SSc characteristics were evaluated. Semiquantitative DUS parameters (resistive index-RI and pulsatility index-PI), were applied for splanchnic vessel assessment in SSc patients and healthy subjects (HS). Moreover, a review and meta-analysis of the literature to understand which the values of the main semiquantitative DUS parameters (RI and PI) are both in SSc patients and HS has been conducted. Seventy-eight patients completed DUS examinations and clinical assessments. 30 (39%) were classified as diffuse cutaneous SSc (dcSSC), 35 (45%) as limited cutaneous SSc (lcSSc) and 13 (17%) as sine scleroderma. A significant difference was found both for SMA RI (p for trend = 0.032) and SMA PI (p for trend = 0.004) between patients with sine scleroderma, lcSSc and dcSSc, with lower values observed in the sine scleroderma and lcSSc groups. IMA RI and PI were significantly correlated with GI symptoms such as fecal incontinence (ῥ - 0.33, p = 0.008 and ῥ - 0.30, p = 0.021, respectively). By multivariate analysis, significant associations were confirmed between SMA RI and SMA PI and mRSS (β 0.248, p = 0.030 and β 2.995, p = 0.004, respectively) and with bosentan (β 0.400, p = 0.003 and β 3.508, p = 0.001, respectively), but not with anticentromere antibody (ACA). No significant differences were found between the weighted median values of SMA RI and SMA PI of SSc patients compared to those of HS that were derived from the meta-analysis of the literature (p = 0.72 and p = 0.64, respectively). This cross-sectional study confirms that the splanchnic vasculature of SSc patients can noninvasively been studied with DUS. Vascular splanchnic involvement correlates with the presence and/or severity of specific clinical features in SSc, including GI. Larger and prospective studies are needed to confirm these preliminary observations and to examine the role of DUS in SSc-risk stratification and GI progression and to obtain definitive data regarding both HS and SSc patients splanchnic DUS parameters., (© 2024. The Author(s).)
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- 2024
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8. The Role of Adipokines between Genders in the Pathogenesis of Osteoarthritis.
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Economou A, Mallia I, Fioravanti A, Gentileschi S, Nacci F, Bellando Randone S, Lepri G, and Guiducci S
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- Humans, Male, Female, Sex Factors, Leptin metabolism, Leptin blood, Sex Characteristics, Resistin blood, Resistin metabolism, Osteoarthritis metabolism, Osteoarthritis blood, Adipokines metabolism, Adipokines blood
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Osteoarthritis (OA) is a chronic, progressive, degenerative joint disease characterized by joint pain, stiffness, and limited movement. It presents significant intra- and inter-individual variability-in particular, between genders. Recent research has increasingly focused on the role of adipokines-especially leptin, adiponectin, and resistin-in the development of OA. Adipokines, peptide hormones primarily secreted by adipose tissue, are involved in crucial physiological processes related to metabolism and immunity. They can also impact bone and cartilage turnover by interacting with joint cells such as osteoblasts, osteoclasts, chondrocytes, and mesenchymal stem cells, thereby linking inflammation with bone cartilage homeostasis. This review aims to elucidate the structure and functions of various adipokines, their serum and synovial levels, and their association with clinical presentation and radiographic progression in OA patients, with a focus on differences between sexes. A narrative literature review was conducted using three databases specifically analyzing sex differences. OA patients generally show elevated serum and synovial levels of leptin, chemerin, and visfatin, as well as high plasma levels of resistin and visfatin. In contrast, synovial levels of adiponectin and omentin are reduced in OA patients compared to healthy individuals, with an inverse relationship to disease severity, suggesting a potential protective role. Resistin and leptin were positively correlated with pain severity and radiographic progression, while adiponectin's role in OA remains controversial. Regarding sex differences, male OA patients exhibited higher serum levels of leptin, chemerin, and omentin compared to healthy controls, with a positive correlation to the BMI and estrogen levels, potentially explaining the sexual dimorphism observed in this condition. Studies on visfatin and lipocalin did not reveal significant differences in synovial or serum levels between the sexes. The role of resistin remains controversial. Adipokines influence the joint microenvironment and contribute to the progression of osteoarthritis (OA). However, the precise biological mechanisms are not yet fully understood due to the complex interactions between the metabolic, mechanical, and immune systems. Further research is needed to clarify their roles in OA and to identify targeted therapies for managing this degenerative disease.
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- 2024
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9. Evidence for telemedicine heterogeneity in rheumatic and musculoskeletal diseases care: a scoping review.
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El Aoufy K, Melis MR, Magi CE, Bellando-Randone S, Tamburini M, Bandini G, Moggi-Pignone A, Matucci-Cerinic M, Bambi S, and Rasero L
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- Humans, Rheumatology methods, Telemedicine, Rheumatic Diseases therapy, Musculoskeletal Diseases therapy
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Telemedicine and digital health represent alternative approaches for clinical practice; indeed, its potential in healthcare services for prevention, diagnosis, treatment, rehabilitation, and disease monitoring is widely acknowledged. These are all crucial issues to consider when dealing with chronic Rheumatic and Musculoskeletal Diseases (RMDs). The aim was to determine the current state of telemedicine in the field of rheumatology, considering the tools and devices in use as well as the Patient Reported Outcomes. A scoping review was performed following the PRISMA-ScR, retrieving articles through five databases from 1990 to 2022. Inclusion criteria were as follows: (I) adult patients with RMDs, (II) original research papers in the English language with available abstracts, and (III) telehealth and telemedicine are provided as healthcare services. Within the 62 included studies, multiple tools of telemedicine were used: 21/62 websites/online platforms, 18/62 mobile applications, 16/62 telephone contacts, 5/62 video-consultations, and 1/62 wearable devices. Outcomes were classified based on the economic, clinical, and humanistic framework. Clinical outcomes assessed through digital tools were pain, disease activity, and serum uric acid levels. Humanistic outcomes have been grouped according to four categories (e.g., mental and physical function, health management, and health perception). The heterogeneity of digital tools in the field of rheumatology highlights the challenge of implementing reliable research into clinical practice. Effective telerehabilitation models have been presented, and the use of a tight control strategy has also been mentioned. Future research should focus on establishing studies on other RMDs as well as summarizing and formulating clinical guidelines for RMDs. Key Points • Evidence for the usefulness of telemedicine and digital health for managing and monitoring rheumatic and musculoskeletal diseases is progressively increasing. • Several digital tools effectively measure clinical and humanistic and patient reported outcomes in rheumatic and musculoskeletal diseases. • Integrating diverse digital tools in rheumatology is challenging yet promising. • Future research should focus on developing standardized recommendations for practical use of telemedicine in daily practice., (© 2024. The Author(s).)
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- 2024
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10. Towards a comprehensive approach to the management and prognosis of systemic sclerosis's patients: The role of comorbidities in the SPRING-SIR registry.
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Orlandi M, Bellando-Randone S, De Angelis R, Ferri C, Giuggioli D, Cacciapaglia F, Magnani L, Cuomo G, Gigante A, Codullo V, Campochiaro C, Ariani A, Foti R, Guiducci S, Matucci-Cerinic M, and Bruni C
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Objectives: The current knowledge about the role of comorbidities in systemic sclerosis (SSc) is limited. Therefore, the aim of this study was to evaluate the prevalence of comorbidities and their impact on disease activity and prognosis in the Systemic sclerosis PRogression INvestiGation (SPRING) registry., Methods: SSc patients from the SPRING registry, fulfilling the ACR/EULAR 2013 classification criteria, with complete data on baseline comorbidities were enrolled. The Charlson comorbidity index (CCI) was used to quantify the overall comorbidity burden. The disease activity was calculated using the revised EUSTAR activity index (AI). The impact of SSc features on CCI, the effect of CCI on SSc disease activity and mortality were tested with multivariable regression models., Results: Among 1910 SSc patients enrolled, 67.3 % had at least one comorbidity at baseline. The most frequent comorbidities were systemic arterial hypertension (23.7 %), osteoporosis (12.9 %) and dyslipidemia (11 %). The mean value of CCI score was 2.0 ± 1.8. When patients were grouped according to increasing levels of CCI, a clear separation in the distribution of SSc-related clinical features could be observed. Among over 900 patients with available follow-up, no association between baseline CCI and changes in disease activity was observed. Conversely, the risk of death over time was independently predicted by both CCI and AI., Conclusions: Comorbidities and disease activity independently impact on the prognosis of SSc patients. This suggests that the management of comorbidities, together with the reduction of disease activity, is fundamental to improve patient survival., Competing Interests: Declaration of competing interest Orlandi M, Bellando-Randone S, Ferri C, Foti R, Codullo V, De Angelis R, Cuomo G, Campochiaro C, Luca Magnani, Cacciapaglia F, Ariani A, Giuggioli D, and Guiducci S have no conflict of interest to declare. Matucci-Cerinic M received fees from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche. Bruni C received consulting fees and/or honoraria from Eli-Lilly, Boehringer Ingelheim, Research grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), Scleroderma Clinical Trials Consortium (SCTC), Scleroderma Research Foundation (SRF), Novartis foundation for biomedical research. Congress supports from Boehringer-Ingelheim. Educational grants from AbbVie and Wellcome Trust., (Copyright © 2024. Published by Elsevier B.V.)
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- 2024
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11. The Potential Role of Butyrate in the Pathogenesis and Treatment of Autoimmune Rheumatic Diseases.
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Coccia C, Bonomi F, Lo Cricchio A, Russo E, Peretti S, Bandini G, Lepri G, Bartoli F, Moggi-Pignone A, Guiducci S, Del Galdo F, Furst DE, Matucci Cerinic M, and Bellando-Randone S
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The gut microbiota is a complex ecosystem of microorganisms residing in the human gastrointestinal tract, playing a crucial role in various biological processes and overall health maintenance. Dysbiosis, an imbalance in the composition and function of the gut microbiota, is linked to systemic autoimmune diseases (SAD). Short-chain fatty acids (SCFAs), especially butyrate, produced by the gut microbiota through the fermentation of dietary fibers, play a significant role in immunomodulation and maintaining intestinal homeostasis. Butyrate is essential for colonocyte energy, anti-inflammatory responses, and maintaining intestinal barrier integrity. Studies show reduced butyrate-producing bacteria in SAD patients, suggesting that increasing butyrate levels could have therapeutic benefits. Butyrate's anti-inflammatory effects and its potential therapeutic role have been studied in rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, systemic sclerosis, and Behçet's disease. Despite promising in vitro and animal model results, human studies are limited, and the optimal strategies for modulating dysbiosis in SADs remain elusive. This review explores the current evidence on the immunoregulatory role of butyrate and its potential therapeutic effects in SAD.
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- 2024
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12. Systemic sclerosis and environment: an intriguing and still debated association.
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Lepri G, Bellando Randone S, Damiani A, Blagojevic J, and Guiducci S
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- Humans, Gene-Environment Interaction, Genetic Predisposition to Disease, Risk Factors, Environmental Exposure adverse effects, Environment, Fibrosis, Scleroderma, Systemic immunology
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Systemic sclerosis (SSc) is characterised by a heterogeneous clinical expression probably reflecting the different genetic background of each patient. Progress has been made in the definition of the principal pathogenetic events of the disease that can be summarised in endothelial damage and dysfunction, inflammation with activation of immune system and fibrosis. The aetiology of the disease still remains to be clarified and probably the first events are attributable to the repeated action of environmental stimuli in genetically predisposed subjects.The aim of the present manuscript is to review the most recent and relevant data regarding the association of SSc with environmental factors.
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- 2024
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13. A multi-national survey to identify clinicians' perspectives concerning Proton Pump inhibitors in patients with systemic sclerosis.
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Bandini G, Alunno A, Oliveira Pinheiro F, Campochiaro C, Galetti I, Matucci Cerinic P, Ruaro B, Moggi Pignone A, Bellando Randone S, Dagna L, Matucci Cerinic M, McMahan ZH, and Hughes M
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- Humans, Surveys and Questionnaires, Male, Female, Proton Pump Inhibitors therapeutic use, Scleroderma, Systemic drug therapy, Gastroesophageal Reflux drug therapy, Attitude of Health Personnel, Practice Patterns, Physicians' statistics & numerical data
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Objectives: Proton Pump Inhibitors (PPIs) are widely used in SSc for gastroesophageal reflux disease (GERD). However, there is little evidence to support their empirical use and long-term safety has been questioned. Our objective was to better describe clinicians' attitudes toward PPIs prescription and use in SSc patients., Methods: Clinicians involved in the care of SSc patients were invited through international physician networks and social media to participate in an online survey., Results: Responses from 227 clinicians from 36 countries were evaluable. The majority 'agreed' (41.4 %) or 'strongly agreed' (45.4 %) that GERD is a major cause of morbidity in SSc. Lifestyle modifications are seldom (16 %) considered effective. Only half 'agreed' (43 %) or 'strongly agreed' (11 %) there is solid evidence supporting PPIs efficacy in SSc. The most common reasons for PPIs prescription were symptomatic GERD unresponsive to lifestyle modification (95 %), objective evidence of GERD (82 %), and hoarseness or respiratory symptoms (71 %). There are variable concerns about PPIs long-term safety in SSc. The three highest (mean) reasons (0-10, here 10 is 'very concerned') were: small intestinal bacterial overgrowth (5.5), osteoporosis (5.4), and drug interactions (5.2). There are significant differences in attitudes towards surgery for refractory GERD, and concerns about potential complications. PPIs may have a putative role for disease modification (e.g., ILD and calcinosis), and the role of immunosuppression is uncertain for GI (gastrointestinal) disease in SSc., Conclusion: PPIs are frequently prescribed in SSc. Side effects are a recognized concern, especially regarding long-term therapy. There is significant variation in attitudes towards surgical intervention. Future research and practical treatment recommendation for PPIs in SSc are urgently needed., Competing Interests: Declaration of competing interest None declared by any of the authors., (Copyright © 2024. Published by Elsevier Inc.)
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- 2024
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14. Can Combination Therapy with Endothelin Receptor Antagonist and PDE5 Inhibitors Prevent Echocardiographic Findings Suspicious for Pulmonary Arterial Hypertension? Description of a Real-Life Case Series.
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Damiani A, Lepri G, Bonomi F, Fiorentini E, Peretti S, Blagojevic J, Bellando Randone S, and Guiducci S
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Objective: To retrospectively evaluate the incidence rate (IR) of elevated echocardiographic estimated systolic pulmonary artery pressure (sPAP), suspected for pulmonary hypertension (PH), in systemic sclerosis (SSc) patients after the introduction of a combination therapy with bosentan and sildenafil for treatment or prevention of digital ulcers., Methods: Patients attending the Scleroderma Unit of the Universital Hospital of Careggi from July 2010 to July 2023 were enrolled. Patients older than 18 years old with a history of digital ulcers, treated with bosentan and sildenafil in combination for at least 12 months, were included. Patients with a diagnosis of PH preceding the introduction of the therapy were excluded. Demographical data, disease duration, laboratoristic, and instrumental data (pulmonary function tests, echocardiographic estimation of sPAP, and ultrasonographic value of renal resistive index) were collected. The IR of echocardiographic signs suspected of pulmonary hypertension and their 95% confidence interval were calculated in events/1000 patients-years., Results: Thirty-five patients were enrolled; the mean disease duration was 12.82 years (SD 5.92). The mean duration of the combination treatment was 81.03 (SD 43.1.3) months, and the total at-risk time was 2674 months. Two patients (5.7%) presented echocardiographic signs of PH (sPAP 50 mmHg and 40 mmHg); the IR was calculated to be 9/1000 patients-years (95% CI 7.95-10.12). In one of the two patients, right heart catheterism (RHC) excluded PAH, while the other patient refused to undergo RHC, and PAH could not be confirmed/excluded. The stability of PFTs and echocardiographic sPAP was observed during the observation time., Conclusions: The results of this retrospective study suggest that combination therapy with endothelin receptor antagonists and phosphodiesterase-5 (PDE5) inhibitors could help in preventing PAH in SSc; prospective case-control studies on a larger population are needed to improve knowledge in this field., Competing Interests: The authors declare no conflicts of interest.
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- 2024
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15. The Burden of Interstitial Lung Involvement in Rheumatoid Arthritis: Could Lung Ultrasound Have a Role in Its Detection? A Literature Review.
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Lepri G, Markovic M, Bellando-Randone S, Sebastiani M, and Guiducci S
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Lung involvement represents a fearful complication in rheumatoid arthritis (RA), potentially involving all compartments of the pulmonary system. Regarding interstitial lung disease (ILD), the HRCT represents the gold standard technique for its diagnosis; however, the examination is burdened by radiation exposure and high costs. In addition, although some risk factors for ILD are known, no algorithms exist to know which patients to submit to HRCT and when. In this context, lung ultrasound (LUS) showed promising results for at least 10 years, demonstrating correlation with high resolution computed tomography (HRCT) findings in other rheumatic diseases. Here, LUS may represent a screening test providing additional information to clinical examination and pulmonary function tests. The data deriving from LUS experience in other rheumatic diseases could steer the future towards the use of this technique also in RA patients, and in this review, we report the most relevant literature regarding LUS in RA-ILD.
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- 2024
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16. Efficacy of physiotherapy treatment in medium and long term in adults with fibromyalgia: an umbrella of systematic reviews.
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Carrasco-Vega E, Guiducci S, Nacci F, Bellando Randone S, Bevilacqua C, Gonzalez-Sanchez M, and Barni L
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- Humans, Treatment Outcome, Time Factors, Adult, Systematic Reviews as Topic, Fibromyalgia therapy, Fibromyalgia physiopathology, Fibromyalgia rehabilitation, Fibromyalgia diagnosis, Physical Therapy Modalities
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Objectives: To summarise the available evidence and assess the effectiveness of medium and long-term physiotherapy treatment in adults with fibromyalgia (FM)., Methods: This systematic review was registered in PROSPERO: CRD42023388356. The databases searched were MEDLINE, PEDro, Scopus, Cinhal, LatinIndex, and Cochrane, using the following keywords: "fibromyalgia", "physiotherapy", "treatment", "therapeutic exercise", "TENS", "laser therapy" and "manual therapy." The included articles analysed treatments with active or passive physiotherapy approaches in patients with FM. The variables included structural characteristics, such as: author, publication year, research question, and main outcome variables. The data on the findings of the articles comprised the following aspects: number of participants, intervention, follow-up, results, and principal conclusions., Results: Thirty-three articles were analysed, with an overall PRISMA score of 18.63±3.36. The active treatment methods analysed were: movement and body awareness therapies (stretching, tai chi, yoga and Pilates); hydrotherapy; physical or aerobic exercise; and multidisciplinary therapy. The passive therapies analysed were: manual therapy; repetitive transcranial magnetic stimulation (rTMS); and other therapies (hyperbaric oxygen therapy, vibration therapy, virtual reality, transcutaneous electric nervous stimulation (TENS), pain neuroscience education, and acupuncture). Evidence was found on the positive effect of physiotherapy treatment on the signs and symptoms of fibromyalgia, such as pain, impairment of physical capacity and worse quality of life., Conclusions: The effectiveness of the active and passive therapies analysed in the management of the symptoms and signs of the disease was positive in most of the studies. However, more specific descriptions of the treatment protocol, frequency, intensity and treatment dose are required to reach a consensus, as well as primary studies for a more extended follow-up period to better evaluate long-term effects.
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- 2024
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17. The Rising Challenge of Poor Health Literacy of Patients with Systemic Sclerosis: Preliminary Data Identify Important Unmet Needs in an Italian Cohort.
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El Aoufy K, Melis MR, Iovino P, Bambi S, Lorini C, Bonaccorsi G, Galetti I, Garbagnati C, Canziani P, Tonolo S, Mitola M, Guiducci S, Furst DE, Matucci-Cerinic M, Rasero L, and Bellando-Randone S
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Rationale and Aim: Health literacy (HL) is pivotal for the successful self-management of chronic diseases. Little HL information is currently available in SSc patients; therefore, the present study aims at evaluating the HL levels in an Italian cohort of SSc patients., Methods: SSc patients were enrolled with the support of Italian patient associations, from September 2022 to March 2023. Health literacy characteristics were derived from the Health Literacy Scale European Questionnaire-16 (HLS-EU-Q16), consisting of 16 items designed on a four-point Likert scale ranging from "very difficult" to "very easy", and three HL levels were identified: inadequate HL (0-8 score); problematic HL (9-12 score); and sufficient HL (13-16 score)., Results: Enrolled patients (n = 57, mean age = 59 years, SD = 13.2) were mostly female (98.2%), partnered (73.7%), and unemployed or retired (67.9%). Almost half of SSc patients were diagnosed more than 10 years ago, with first symptoms appearing on average 19 years ago (SD 10.5). In 63% of the participants, the overall health literacy skills were inadequate, or problematic, especially in the health care and disease prevention domains. Indeed, 49.2% of the patients declared difficulty in finding information on treatments for illnesses and where to get professional help (42.1%), 47.6% found difficulty in retrieving information on how to manage mental health problems, and 40.4% declared difficulties in judging whether the information on health risks in the media was reliable., Conclusions: Our findings show that SSc patients have inadequate or problematic levels of HL, suggesting the need for periodic screenings to uncover poor health literacy skills and to provide tailored and understandable educational material. This study was not registered.
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- 2024
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18. Patients' unmet needs and treatment preferences concerning digital ulcers in systemic sclerosis.
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Bandini G, Alunno A, Alcacer-Pitarch B, Ruaro B, Galetti I, El-Aoufy K, Pinheiro F, Campanaro G, Jade J, Di Donato S, Muir L, Moggi Pignone A, Bellando Randone S, Del Galdo F, McMahan ZH, Matucci-Cerinic M, and Hughes M
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Introduction: Digital ulcers (DUs) significantly impact on quality of life and function in patients with systemic sclerosis (SSc). The aim of our survey was to explore patients' perspectives and their unmet needs concerning SSc-DUs., Materials: SSc patients were invited through international patient associations and social media to participate in an online survey., Results: 358 responses were obtained from 34 countries: US (65.6%), UK (11.5%) and Canada (4.5%). Recurrent DUs are common: >10 DUs (46.1%), 5-10 DUs (21.5%), 1-5 DUs (28.5%), 1 DU (3.9%). Fingertip DUs were most frequent (84.9%), followed by those overlying the interphalangeal joints (50.8%). The impact of DUs in patients is broad, from broad-ranging emotional impacts to impact on activities of daily living, and personal relationships. Half (51.7%) of respondents reported that they received wound/ulcer care, most often provided by non-specialist wound care clinics (63.8%). There was significant variation in local (wound) DU care, in particular the use of debridement and pain management. DU-related education was only provided to one-third of patients. One-quarter (24.6%) were 'very satisfied' or 'satisfied' that the provided DU treatment(s) relieved their DU symptoms. Pain, limited hand function, and ulcer duration/chronicity were the main reasons for patients to consider changing DU treatment., Conclusions: Our data show that there is a large variation in DU treatment between countries. Patient access to specialist wound-care services is limited and only a small proportion of patients had their DU needs met. Moreover, patient education is often neglected. Evidence-based treatment pathways are urgently needed for DU management., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2024
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19. Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case-control study from the Italian national multicenter "SPRING" (Systemic Sclerosis Progression InvestiGation) Registry.
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Riccieri V, Pellegrino G, Cipolletta E, Giuggioli D, Bajocchi G, Bellando-Randone S, Dagna L, Zanframundo G, Foti R, Cacciapaglia F, Cuomo G, Ariani A, Rosato E, Lepri G, Girelli F, Zanatta E, Bosello SL, Cavazzana I, Ingegnoli F, De Santis M, Murdaca G, Abignano G, Romeo N, Della Rossa A, Caminiti M, Iuliano A, Ciano G, Beretta L, Bagnato G, Lubrano E, De Andres I, Giollo A, Saracco M, Agnes C, Lumetti F, Spinella A, Magnani L, Campochiaro C, De Luca G, Codullo V, Visalli E, Di Vico C, Gigante A, Saccon F, Grazia Lazzaroni M, Franceschini F, Generali E, Mennillo G, Barsotti S, Pagano Mariano G, Calabrese F, Furini F, Vultaggio L, Parisi S, Peroni CL, Bianchi G, Conti F, Cozzi F, D'Angelo S, Doria A, Fusaro E, Govoni M, Guiducci S, Iannone F, Salvarani C, Sebastiani GD, Ferri C, Matucci-Cerinic M, and De Angelis R
- Abstract
Background: Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data., Patients and Methods: Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group)., Results: Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, " late " scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis., Conclusion: Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies in the different centers suggests the need of a rational therapeutical approach based on the clinical characteristics of different patients' subsets., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)
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- 2024
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20. The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis-Interstitial Lung Disease in the European Scleroderma Trial and Research Database.
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Lepri G, Bruni C, Tofani L, Moggi-Pignone A, Orlandi M, Tomassetti S, Hughes M, Del Galdo F, Irace R, Distler O, Riccieri V, Allanore Y, Gheorghiu AM, Siegert E, De Vries-Bouwstra J, Hachulla E, Tikly M, Damjanov N, Spertini F, Mouthon L, Hoffmann-Vold AM, Gabrielli A, Guiducci S, Matucci-Cerinic M, Furst D, Bellando-Randone S, and Eustar Collaborators
- Abstract
Background and Objectives: In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD., Methods: Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t
0 ) and after 12 (±4) (t1 ) and 24 (±4) (t2 ) months., Results: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t2 . Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t2 ( p = 0.0031). Neither the mean t0 to t1 change (Δ) of DLCO nor the mean t0 to t1 FVCΔ predicted the appearance of ILD at t2 . Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (±4) months, we observed a moderate predictive capability of t0 DLCO < 80%, stronger than that of FVC < 80%., Conclusions: We suggest that an impaired baseline DLCO may be predictive of the appearance of ILD after 2 years of follow-up. This result advances the hypothesis that a reduction in gas exchange may be considered an early sign of lung involvement. However, further rigorous studies are warranted to understand the predictive role of DLCO evaluation in the course of SSc.- Published
- 2024
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21. The differential crosstalk of the skin-gut microbiome axis as a new emerging actor in systemic sclerosis.
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Russo E, Bellando-Randone S, Carboni D, Fioretto BS, Romano E, Baldi S, El Aoufy K, Ramazzotti M, Rosa I, Lepri G, Di Gloria L, Pallecchi M, Bruni C, Melchiorre D, Guiducci S, Manetti M, Bartolucci GL, Matucci-Cerinic M, and Amedei A
- Subjects
- Humans, Feces, Skin, Gastrointestinal Microbiome, Scleroderma, Systemic, Gastrointestinal Diseases
- Abstract
Objectives: We characterized the microbiota in SSc, focusing on the skin-oral-gut axis and the serum and faecal free fatty acid (FFA) profile., Methods: Twenty-five SSc patients with ACA or anti-Scl70 autoantibodies were enrolled. The microbiota of faecal, saliva and superficial epidermal samples was assessed through next-generation sequencing analysis. GC-MS was used to quantify faecal and serum FFAs. Gastrointestinal symptoms were investigated with the University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument (UCLA GIT-2.0) questionnaire., Results: The ACA+ and anti-Scl70+ groups displayed different cutaneous and faecal microbiota profiles. The classes of cutaneous Sphingobacteriia and Alphaproteobacteria, the faecal phylum Lentisphaerae, the levels of the classes Lentisphaeria and Opitutae, and the genus NA-Acidaminococcaceae were significantly higher in faecal samples from the ACA+ patients than in samples from the anti-Scl70+ patients. The cutaneous Sphingobacteria and the faecal Lentisphaerae were significantly correlated (rho = 0.42; P = 0.03). A significant increase in faecal propionic acid was observed in ACA+ patients. Moreover, all levels of faecal medium-chain FFAs and hexanoic acids were significantly higher in the ACA+ group than in the anti-Scl70+ group (P < 0.05 and P < 0.001, respectively). In the ACA+ group, the analysis of the serum FFA levels showed an increasing trend in valeric acid., Conclusion: Different microbiota signatures and FFA profiles were found for the two groups of patients. Despite being in different body districts, the cutaneous Sphingobacteria and faecal Lentisphaerae appear interdependent., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2024
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22. Advanced and traditional chest MRI sequence for the clinical assessment of systemic sclerosis related interstitial lung disease, compared to CT: disease extent analysis and correlations with pulmonary function tests.
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Landini N, Orlandi M, Calistri L, Nardi C, Ciet P, Bellando-Randone S, Guiducci S, Benkert T, Panebianco V, Morana G, Matucci-Cerinic M, and Colagrande S
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- Humans, Lung diagnostic imaging, Tomography, X-Ray Computed, Magnetic Resonance Imaging, Respiratory Function Tests, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial etiology, Scleroderma, Systemic complications, Scleroderma, Systemic diagnostic imaging
- Abstract
Background: MRI is a radiation-free emerging alternative to CT in systemic sclerosis related interstitial lung disease (SSc-ILD) assessment. We aimed to compare a T2 radial TSE and a PD UTE MRI sequence with CT in SSc-ILD extent evaluation and correlations with pulmonary function tests (PFT)., Material and Methods: 29 SSc-ILD patients underwent CT, MRI and PFT. ILD extent was visually assessed. Lin's concordance correlation coefficients (CCC) and Kruskal Wallis test (p-value < 0.05) were computed for inter-method comparison. Patients were divided in limited and extended disease, defining extended ILD with two methods: (A) ILD>30% or 10%
20% or 20% with FVC%<70%. MRI Sensitivity, Specificity, Positive Predictive Value (PPV), Negative Predictive Value (NPV) and Accuracy were assessed. Pearson correlation coefficients r (p-value<0.025) were computed between ILD extents and PFT (FVC% and DLCO%)., Results: Median ILD extents were 11%, 11%, 10% on CT, radial TSE and UTE, respectively. CCC between CT and MRI was 0.95 for both sequences (Kruskal-Wallis p-value=0.64). Sensitivity, Specificity, PPV, NPV and Accuracy in identifying extended disease were: (A) 87.5 %, 100 %, 100 %, 95.5 and 96.6 % with radial TSE and 87.5 %, 95.2 %, 87.5 %, 95.2 and 93.1 % with UTE; (B) 86.7 %, 86.4 %, 66.7 %, 95.0 % and 86.2 % for both sequences. Pearson r of CT, radial TSE and UTE ILD extents with FVC were -0.66, -0.60 and -0.68 with FVC, -0.59, -0.56 and -0.57 with DLCO, respectively (p<0.002)., Conclusions: MRI sequences may have similar accuracy to CT to determine SSc-ILD extent and severity, with analogous correlations with PFT., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.) - Published
- 2024
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