Publications by authors named "Marilia A Dagostin"
Article Synopsis
- This study evaluated the effectiveness of a systemic score in predicting severe outcomes in patients with Still disease, including the risk of mortality and severe complications like macrophage activation syndrome.
- Involving 597 patients, the study found that a higher systemic score significantly correlated with life-threatening outcomes, with scores of 7 or above indicating a greater risk.
- Key components that were most predictive of severe outcomes included liver and lung involvement, emphasizing the importance of these factors in managing Still disease.
Correction: Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.
Intern Emerg Med
January 2024
Efficacy of canakinumab in patients with Still's disease across different lines of biologic therapy: real-life data from the International AIDA Network Registry for Still's Disease.
Front Med (Lausanne)
December 2023
Introduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment.
Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease.
Article Synopsis
- - This study investigates whether pediatric-onset, adult-onset, and elderly-onset Still's disease are the same condition or different diseases by comparing demographic, clinical, and treatment response data across these age groups.
- - Out of 411 patients surveyed, most were adults (76.4%), while 15.8% were pediatric and 7.8% were elderly, with significant differences found in symptoms like skin rash and arthritis being more prevalent in children, and pleuritis in the elderly.
- - Overall, while some minor differences in symptoms and lab results were noted among the age groups, the study concludes that Still's disease has similar demographic and treatment characteristics across pediatric, adult, and elderly patients.
Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry.
Intern Emerg Med
November 2023
Article Synopsis
- The study focuses on patients with Still's disease to identify clinical and laboratory signs associated with macrophage activation syndrome (MAS), a serious complication.
- Out of 414 patients examined, 39 (9.4%) developed MAS, with several significant variables like liver involvement and high ferritin levels linked to its occurrence.
- Multivariate analysis revealed that having hepatomegaly and monoarthritis were directly associated with MAS, while factors like a normal platelet count were protective against its development.
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.
Objective: To evaluate inactivated CoronaVac prime vaccination, antibody decay, booster dose, and safety in ANCA-Associated Vasculitis (AAV) patients.
Methods: Fifty-three AAV patients and 106 Controls (CG) received CoronaVac on days: D0 (first dose), D28(second dose), and D210 (booster dose, 32 AAV: 32 CG). The primary outcome was immunogenicity after the second vaccine dose (day 69) assessed by Seroconversion Rates (SC) of anti-SARS-CoV-2 S1/S2 IgG and Neutralizing Antibodies (NAb).
Background/objectives: Long-term efficacy and safety of tocilizumab (TCZ) in adult-onset Still's disease (AOSD) mostly derive from small case series. Herein we report a registry-based study investigating TCZ efficacy and safety in a cohort of patients with AOSD evaluated by clinical and serum inflammatory markers as well as drug retention rate analysis.
Methods: This is an international multicentre study analyzing data from patients with AOSD regularly enrolled in the AIDA registry.
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.
Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) international Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination.
Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients' management.
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.
View Article and Find Full Text PDFObjective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder.
Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management.
Article Synopsis
- The paper discusses the creation of the AIDA International Registry, aimed at tracking data for pediatric and adult patients with non-infectious uveitis.
- This registry collects a wide range of standardized information through a digital platform called REDCap, focusing on patient demographics, clinical data, and treatment details to enhance research quality.
- As of late 2021, 95 centers from 19 countries are participating, and the registry is expected to help in global research collaborations and improve understanding of non-infectious uveitis.
Article Synopsis
- - The article discusses the creation and implementation of a global registry by the AIDA Network to gather data from both pediatric and adult patients with immune-mediated scleritis.
- - Using the REDCap tool, the registry collects a wide range of data securely, while allowing for adaptability as scientific knowledge grows and enabling collaboration with other registries for sustainability.
- - Since its inception, the registry has engaged 99 centers across 20 countries, collecting comprehensive data to enhance research and improve clinical management of this rare ocular inflammatory condition.
The mortality rate of patients with anti-neutrophil cytoplasm antibody -associated vasculitis (AAV) is higher than the general population. To date, no studies have evaluated the factors associated with unfavorable outcomes in Brazilian patients, who represent a miscegenated population. Our objective was to identify clinical and laboratory features associated with mortality in Brazilian patients with AAV.
View Article and Find Full Text PDF