Publications by authors named "Matucci-Cerinic M"
Objectives: The 2022 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines for pulmonary arterial hypertension (PAH) recommend risk stratification to optimize management. However, the performance of generic PAH risk stratification tools in patients with systemic sclerosis (SSc)-associated PAH remains unclear. Our objective was to identify the most accurate approach for risk stratification at SSc-PAH diagnosis.
View Article and Find Full Text PDFObjectives: To update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for treatment of systemic sclerosis (SSc), incorporating new evidence and therapies.
Methods: An international task force was convened in line with EULAR standard operating procedures. A nominal group technique exercise was performed in two rounds to define questions underpinning a subsequent systematic literature review.
Background: Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), and is associated with a poor prognosis. Therapeutic strategies to treat SSc-pHI are not yet defined.
Objectives: To evaluate the efficacy of immunosuppressive therapy on cardiac magnetic resonance (CMR) features in patients with CMR-proven SSc-pHI.
Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included.
Objective: Systemic sclerosis Impact of Disease (ScleroID) is the first comprehensive patient-reported outcome measure (PROM) specifically developed for systemic sclerosis (SSc). We investigated the performance of ScleroID in patients with diffuse cutaneous SSc (dcSSc), as a prerequisite for its use in randomised controlled trials (RCTs) testing potentially disease-modifying drugs.
Methods: All patients with dcSSc from the large, multicentric, ScleroID cohort were included.
Article Synopsis
- Systemic lupus erythematosus (SLE) leads to immune dysfunction and increased infection susceptibility, often resulting in infection-associated flares (IAFs) that can resolve alongside infections.
- In a study of 2,039 visits, researchers identified 134 flares (17% yearly deterioration of lupus low disease activity state [LLDAS]), with 38 classified as IAFs primarily linked to bacterial and herpesvirus infections.
- IAFs showed similar or greater severity in symptoms compared to other flares, with distinct characteristics such as lower anti-DNA antibody levels in viral IAFs, indicating the need for further research to explore these differences and improve treatment approaches.
Objectives: In systemic sclerosis (SSc), gastrointestinal involvement is one of the earliest events. We compared the gut microbiota (GM), its short-chain fatty acids (SCFAs) and host-derived free fatty acids (FFAs) in patients with very early diagnosis of SSc (VEDOSS) and definite SSc.
Methods: Stool samples of 26 patients with SSc, 18 patients with VEDOSS and 20 healthy controls (HC) were collected.
Objective: To optimise the organisation of care and encourage the adoption of good clinical practices, the RarERN Path methodology was designed within ERN ReCONNET. The aim of our work was to report the application of RarERN Path on systemic sclerosis within the ERN ReCONNET centres, providing a feasible and flexible organisational reference model for optimising the systemic sclerosis care pathway in different countries.
Methods: RarERN Path is a six-phase methodology which enables the creation of a reference organisational model co-designed on the basis of the expertise of different stakeholders.
Article Synopsis
- The study aimed to see if adding oral glucocorticoids to immunosuppressive therapy improves skin conditions and safety in patients with early diffuse cutaneous systemic sclerosis (dcSSc).
- It compared two groups: one receiving glucocorticoids combined with immunosuppression, and the other receiving only immunosuppression, assessing changes in the modified Rodnan skin score (mRSS) over about a year.
- Results showed no significant difference in skin score improvement between the groups, indicating that low-dose glucocorticoids didn't provide added benefits for skin fibrosis nor raised the risk of scleroderma renal crisis.
Systemic sclerosis (SSc) 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 SSc . As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis.
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- Dystrophic calcinosis is a condition where calcified materials accumulate in tissues despite normal calcium and phosphorus levels, commonly seen in systemic sclerosis (SSc) and significantly impacting patient quality of life.
- The management of calcinosis in SSc is challenging due to risks like infection, pain, and the absence of standardized treatment options, with its precise causes still unclear.
- Factors contributing to calcinosis development include cell differentiation, mineralization imbalances, and local disturbances in mineral levels, alongside pathophysiological changes in SSc that may promote its formation, highlighting the need for further research into underlying mechanisms for potential treatment strategies.
Article Synopsis
- - Gastrointestinal complications are common in systemic sclerosis (SSc) and may be linked to autoimmune factors and blood vessel damage, but the exact causes are unclear.
- - The study examined the small blood vessels in the abdomen of SSc patients using Doppler Ultrasound to explore their relationship with GI symptoms and disease progression.
- - Results showed differences in blood flow measurements between types of SSc and found significant correlations between blood vessel function and GI symptoms like fecal incontinence.
Article Synopsis
- A team updated the 2017 EULAR treatment recommendations for systemic sclerosis (SSc) to include new evidence and therapy options.
- They conducted a systematic literature review and developed 22 recommendations across 8 clinical areas, focusing mainly on skin fibrosis and interstitial lung disease (ILD).
- The updated guidelines now suggest new treatments like mycophenolate mofetil and rituximab and emphasize a research agenda for future therapies targeting various aspects of SSc.
Article Synopsis
- The study aimed to create an easy-to-use nomogram for predicting interstitial lung disease (ILD) in patients with idiopathic inflammatory myositis (IIM) based on a large retrospective analysis of 205 patients from Shantou Central Hospital.
- Using LASSO regression, researchers identified four risk factors (age, respiratory symptoms, MDA-5 antibodies, and anti-ARS antibodies) and found that including KL-6 levels and LUS B-lines significantly improved the model's predictive accuracy.
- The final model showed strong predictive performance with an ROC AUC of up to 0.951, and a web version was created to enhance accessibility for clinicians.
Background: Mycophenolate mofetil (MMF) is a mainstay for the treatment of systemic sclerosis (SSc). The occurrence and implications of MMF-related adverse events on drug retention rates in real life remain poorly defined. We aimed to determine the MMF retention rate and to investigate the causes and patterns of discontinuation, adverse events (AEs) and treatment options used after discontinuation.
View Article and Find Full Text PDFSystemic autoimmune rheumatic diseases (SARDs) related pulmonary disease is highly prevalent, with variable clinical presentation and behavior, and thus is associated with poor outcomes and negatively impacts quality of life. Chest high resolution computed tomography (HRCT) is still considered a fundamental imaging tool in the screening, diagnosis, and follow-up of pulmonary disease in patients with SARDs. However, radiation exposure, economic burden, as well as lack of point-of-care CT equipment limits its application in some clinical situation.
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- Systemic sclerosis (SSc) is an autoimmune disease that is often overlooked compared to others like rheumatoid arthritis, and this study examines gene expression and immune cell profiles in patients with SSc.
- * RNA sequencing data from 119 patients and healthy controls revealed 1,148 differentially expressed genes (DEGs) unique to SSc, indicating altered megakaryocyte processes and decreased neutrophil function.
- * The findings point to shared pathogenic pathways in autoimmune diseases, specifically emphasizing megakaryocyte proliferation, and suggest potential new targets for SSc treatment.*
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.
View Article and Find Full Text PDFObjectives: 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.
Patients with systemic autoimmunity due to autoimmune rheumatic diseases (ARDs) or sarcoidosis frequently present with systemic manifestations including cardiac involvement. Cardiac rhythm disturbances and specifically ventricular arrhythmias (VAs) may affect the prognosis of these patients. Cardiovascular magnetic resonance imaging (CMR) is a non-invasive imaging modality that can provide valuable diagnostic and prognostic information in patients with ARDs or systemic autoimmunity in general.
View Article and Find Full Text PDFBackground: Hypothesizing that early treatment yields improved prognosis, we aimed to investigate how the timing of immunosuppressive treatment relates to interstitial lung disease (ILD) development and the course of pulmonary function in systemic sclerosis (SSc).
Methods: A cohort was created using data from the EUSTAR database and Nijmegen Systemic Sclerosis cohort, including adult patients who started their first immunosuppressive treatment (ie mycophenolate mofetil, methotrexate, cyclophosphamide, tocilizumab or rituximab) after SSc diagnosis, and no signs of ILD on high-resolution CT. ILD-free survival and the course of forced vital capacity % predicted (ppFVC) were assessed for up to 5 years follow-up comparing patients who started early (disease duration ≤ 3 years) vs late with immunosuppression.
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.
View Article and Find Full Text PDFObjective: The efficacy of Janus kinase inhibitors (JAKi) in rheumatoid arthritis (RA) has been clearly shown. However, information on comparative drug retention rates (DRRs) of different JAKi is heterogeneous. The aim of this study was to compute and compare DRRs of different JAKi in a large cohort of RA patients.
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