Publications by authors named "Francesca Ruffilli"

Article Synopsis
  • The study aimed to explore gender differences in the phenotypical expression of Behçet's disease (BD) using data from the International AIDA Network Registry, focusing on damage index, disease manifestations, and cardiovascular risk.
  • A total of 1024 patients (567 males and 457 females) were examined, revealing that males had a significantly higher overall damage index and more frequent occurrences of uveitis and vascular involvement, while females showed higher instances of arthralgia, arthritis, and CNS involvement.
  • Key factors associated with major organ involvement included male gender, treatment with biologic agents, origin from endemic regions, and longer disease duration, indicating a more severe course of BD in males compared to females.
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Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

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Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

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Article Synopsis
  • The study examined pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) enrolled in the Italian P-RHEUM.it cohort from 2018 to 2023, focusing on maternal and infant health.
  • Results showed that out of 866 pregnancies, 15.6% experienced maternal disease flares, 30.1% had obstetrical complications, and 91.7% resulted in live births, with a relatively low rate of perinatal deaths and complications.
  • The research concluded that effective preconception counseling and proper medication management during pregnancy may help reduce disease-related risks, suggesting that outcomes for women with ARD were comparable to those in the general obstetric population.
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Behçet's disease (BD) is a heterogeneous multifactorial autoinflammatory disease characterized by a plethora of clinical manifestations. Cutaneous lesions are considered hallmarks of the disease. However, their evolution over time and a thorough description are scarcely reported in non-endemic regions.

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Article Synopsis
  • A study assessed the clinical characteristics and treatment effectiveness of methotrexate (MTX) in patients with Still's disease, focusing on changes in disease activity and inflammatory markers.
  • Among the 171 patients studied, MTX was commonly used with glucocorticoids (GCs), and many showed significant improvement, with 38.6% achieving clinical remission and reduced inflammation.
  • The results suggest that MTX is effective in managing Still's disease symptoms, reducing the need for GCs, though some patients discontinued MTX due to side effects or lack of efficacy.
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Systemic lupus erythematosus (SLE) is characterized by multisystemic clinical manifestations ranging from a relatively mild involvement to potentially life-threatening complications. Due to this complexity, a multidisciplinary (MD) approach is the best strategy for optimizing patients' care. The main aim of this systematic literature review (SLR) was to scrutinize the published data regarding the MD approach for the management of SLE patients.

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Objective: The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.

Methods: This is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries.

Results: Since its launch, 113 centers from 23 countries in 4 continents have been involved.

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Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet's disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved.

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