Publications by authors named "Marco Matucci Cerinic"

Article Synopsis
  • The study evaluates the safety and effectiveness of switching from the original rituximab (RTX-O) to its biosimilars (RTX-B) in patients with autoimmune rheumatic diseases (ARDs), with a focus on long-term drug retention and safety.
  • It included 181 patients who switched to either GP2013 or CT-P10, finding that the drug retention rates were high over 36 months, particularly for GP2013.
  • The transition to CT-P10 showed a higher risk of discontinuation, but overall, the switch to RTX-B was deemed safe with a low incidence of adverse events.
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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.

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Systemic lupus erythematosus (SLE) is characterised by generalised immune dysfunction, including infection susceptibility. Infection-associated flares (IAFs) are common and might rapidly self-resolve, paralleling infection resolution, but their specific clinical phenotype is poorly understood. Therefore, we screened 2039 consecutive visits and identified 134 flares, defined as a loss of the lupus low disease activity state (LLDAS), from 1089 visits at risk spanning over multiple follow-up years, yielding an average yearly LLDAS deterioration rate of 17%.

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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.

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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.

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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.
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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.

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Objective: Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival.

Methods: Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, and valid mRSS at 12±3 months follow up were included.

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Article Synopsis
  • 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.
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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.
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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.
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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.
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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.

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Systemic 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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Article Synopsis
  • 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.*
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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.

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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.

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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.

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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.

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Article Synopsis
  • Scientists wanted to see how smoking affected people with a disease called systemic sclerosis (SSc).
  • They studied over 12,000 patients and found that smoking was linked to worse health outcomes, especially for women.
  • Smokers had a higher risk of serious issues like skin problems and even death compared to non-smokers, especially when they had specific antibodies.
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Objectives: to report real-life data on rituximab retention-rate as indicator of safety and efficacy in a multicentric national cohort of systemic sclerosis patients.

Methods: SSc patients treated with rituximab and followed for at least 36 months were included, clinically characterized, and longitudinally monitored. A competing risk analysis with sub-Hazard Ratio(sHR) definition was performed to explore the clinical variables linked to specific cause of rituximab discontinuation.

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Article Synopsis
  • Psoriasis (PsO) and psoriatic arthritis (PsA) are often not treated adequately, and a multidisciplinary approach is needed, especially with the emergence of anti-TNF biosimilars like adalimumab.
  • A panel of 36 Italian dermatologists and rheumatologists used the Delphi Method to reach a consensus on how to effectively manage PsO and PsA with adalimumab, ultimately showing strong support for its efficacy and long-term use.
  • The study concluded that adalimumab is highly recommended as a first-line treatment for specific cases, such as concurrent conditions like inflammatory bowel disease or uveitis, and serves as a solid alternative after other treatments fail.
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Introduction: Idiopathic inflammatory myopathies (IIM) represent a rare and heterogenous group diseases, and their treatment is not fully defined yet. According to previous small case series, the combination of mycophenolate mofetil (MMF) and rituximab (RTX) may be effective in controlling difficult-to-treat patients. Our aim was to further explore the efficacy and safety of this combined approach in patients with IIM.

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In SSc, dystrophic calcinosis is one of the major clinical manifestations, characterized by the deposition of insoluble calcific substances in tissues, predominantly in the chemical form of calcium hydroxyapatite. Furthermore, calcinosis might lead to compressive neuropathies and severe pain. Current evidence suggests that tissue ischemia and repeated trauma are implicated in the development of calcinosis; however, there are still too many unknown areas that need to be investigated.

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