Publications by authors named "Marilia Ambiel 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.
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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.

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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.
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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.
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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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