Publications by authors named "Tarsia M"
Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
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.
Objectives: (1) characterizing a group of spondyloarthritis (SpA) patients with systemic auto-inflammatory symptoms (S-SpA); (2) comparing SpA features with and without auto-inflammatory symptoms; (3) comparing the auto-inflammatory features of S-SpA and Still's disease (SD).
Methods: Retrospective observational study. Clinical data of adult and pediatric patients with S-SpA, SD or SpA were collected retrospectively and analyzed.
Article Synopsis
- The study investigates the relationship between Familial Mediterranean fever (FMF), a severe inflammatory condition, and the risk of cancer development compared to other diseases like fibromyalgia, Still's disease, and Behçet's disease.
- Results show that FMF patients have a significantly lower risk for malignancies compared to fibromyalgia patients, with a risk ratio (RR) of 0.26; however, this risk is less clear when comparing with the other conditions.
- Factors influencing cancer risk in FMF patients include age at onset and diagnosis, frequency of disease attacks, and treatment with specific biotechnological agents.
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.
Introduction: This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).
Methods: This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled.
Article Synopsis
- Many biological drug patents have expired, leading to the development of biosimilar agents (BIOs), but their use in children raises concerns, prompting a study on their efficacy and safety for treating pediatric non-infectious uveitis (NIU).
- Data collected from pediatric patients treated with TNF inhibitors BIOs showed a significant decrease in flare-ups and ocular complications, as well as a reduction in the need for glucocorticoids during treatment.
- The study included 47 patients, demonstrating strong treatment retention rates and maintaining visual acuity throughout the therapy, while only recording a few minor adverse events.
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.
In recent years, imaging has become increasingly important to confirm diagnosis, monitor disease activity, and predict disease course and outcome in children with juvenile idiopathic arthritis (JIA). Over the past few decades, great efforts have been made to improve the quality of diagnostic imaging and to reach a consensus on which methods and scoring systems to use. However, there are still some critical issues, and the diagnosis, course, and management of JIA are closely related to clinical assessment.
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- 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.
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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- 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.
Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD).
Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry.
Article Synopsis
- The study investigates the effectiveness of adalimumab (ADA), a tumor necrosis factor inhibitor, in treating pediatric patients with non-infectious non-anterior uveitis, where scientific evidence is currently limited.
- Data from the AutoInflammatory Disease Alliance (AIDA) registry were analyzed, involving 21 patients who received ADA treatment for various types of uveitis.
- Results showed significant improvements, with a decrease in ocular inflammation, fewer ocular flares, and reduced glucocorticoid usage, indicating ADA's potential as an effective therapy for these patients.
This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet's Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.
View Article and Find Full Text PDFObjective: To evaluate the potential role of K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction.
Patients And Methods: The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.
The aim of the study is to evaluate the frequency and features of positive pathergy test (PPT) in Italy, its role in the diagnosis of Behçet's disease (BD), and any association with other BD-related manifestations. 52 BD patients, 52 patients with axial spondyloarthritis (ax-SpA), and 26 healthy controls (HCs) underwent intradermal injection of normal saline and intradermal needle soaked with fresh self-saliva. The results of pathergy tests were statistically analysed in the light of demographic, clinical, and therapeutic features of subjects enrolled.
View Article and Find Full Text PDFThe Autoinflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases.
Front Med (Lausanne)
September 2022
Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network.
Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform.
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: Aim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome.
Methods: This is a physician-driven, non-population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from PFAPA patients. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool.
Article Synopsis
- This study examined the effectiveness of biologic drugs in treating pediatric patients with scleritis, assessing data over various timeframes.
- Five patients received treatments including adalimumab and infliximab after failing conventional therapies, demonstrating a significant reduction in inflammation and relapses.
- The findings indicate that biologics not only resolved scleritis in the majority of cases but also enabled the reduction or cessation of glucocorticoid use while preserving vision.