Development and Implementation of the AIDA International Registry for Patients with Non-Infectious Scleritis

Casa, Francesca Della; Vitale, A.; Pereira, Rosa Maria Rodrigues; Guerriero, Silvana; Ragab, Gaafar; Lopalco, Giuseppe; Cattalini, Marco; Mattioli, Irene; Parronchi, Paola; Paroli, Maria Pia; Giudice, Emanuela Del; Gaggiano, Carla; Dagostin, Marília A.; Albano, Valeria; Soliman, Mahmoud; Colella, Sergio; Sota, Jurgen; Antonelli, Isabele P. B.; Alessio, Giovanni; Caggiano, Valeria; Tufan, Abdurrahman; Amin, Rana Hussein; Tarsia, Maria; Ghanema, Mahmoud; Iannone, Florenzo; Ricci, Francesca; Torre, Francesco La; Więsik-Szewczyk, Ewa; Conticini, Edoardo; Gentileschi, Stefano; Dammacco, Rosanna; Cimaz, Rolando; Frediani, Bruno; Abbruzzese, Anna; Ruscitti, Piero; Tosi, Gian Marco; Giordano, Heitor F.; Conforti, Alessandro; Balistreri, Alberto; Rigante, Donato; Cantarini, Luca; Fabiani, Claudia

doi:10.1007/s40123-022-00466-2

Cited by 12 publications

(6 citation statements)

References 14 publications

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“…Regardless of the real impact that this disease has in the population, VEXAS is to be considered a rare disease at present. Therefore, as for other autoinflammatory diseases ( 6 – 8 ), we have developed and launched a Registry capable of gathering the few real-life data available worldwide. While waiting for randomized controlled clinical trials, which are likely to take many years for their conduction, a registry dedicated to VEXAS syndrome can lead to the rapid collection of real-life data from a sufficiently large number of patients.…”

Section: Discussionmentioning

confidence: 99%

“…As for other AIDA registries ( 6 – 8 ), the assessment of the socioeconomic influence of the disease on the national healthcare systems, on patients' social role and job impact represent an intriguing subject of analysis. Other objectives will be identified based on the challenges that clinical practice and scientific research will bring forward in the coming years.…”

Section: Discussionmentioning

confidence: 99%

“…Regarding autoinflammatory diseases, the AutoInflammatory Disease Alliance (AIDA) has already developed and launched eight international registries for patients with many rare autoinflammatory diseases ( 6 – 8 ). The AIDA Project has already allowed the construction of an international Network of physicians and researchers interested in putting together information to expand current evidence about monogenic autoinflammatory diseases, Still's disease, Schnitzler's syndrome, Behçet's disease, periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome, non-infectious uveitis, non-infectious scleritis, and undifferentiated systemic autoinflammatory diseases (USAIDs).…”

Section: Introductionmentioning

confidence: 99%

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Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome

et al. 2022

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ObjectiveThe 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.MethodsThis 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. The Registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, thus enhancing international collaboration and data sharing for research purposes. The Registry is practical enough to be easily modified to meet future needs regarding VEXAS syndrome.ResultsTo date (April 22nd, 2022), 113 Centers from 23 Countries in 4 continents have been involved; 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) are currently able to access the registry for data entry (or data sharing) and collection. The Registry includes 4,952 fields organized into 18 instruments designed to fully describe patient's details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access.ConclusionThis international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease, with the final goal to obtain real-world evidence for daily clinical practice, especially in relation to the comprehension of this disease about the natural history and the possible therapeutic approaches. This Project can be found on https://clinicaltrials.gov NCT05200715.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome

et al. 2022

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“…The Registry for Schnitzler's syndrome is classified as a clinical-, physician-driven, population- and electronic-based registry; it was developed along with other autoinflammatory disease-specific registries in the context of AIDA network ( 7 – 9 ).…”

Section: Methodsmentioning

confidence: 99%

Development and implementation of the AIDA international registry for patients with Schnitzler's syndrome

et al. 2022

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ObjectiveThe present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.MethodsThis 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.ResultsSince its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access.ConclusionsThis International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder.

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“…In addition to pathognomonic joint disease, JIA is also burdened by extra-articular involvement and comorbidities ( 4 – 6 ), the most frequent of which are uveitis and cardiovascular complications ( 7 – 9 ). However, the pathogenesis of renal involvement in JIA is poorly understood, it may develop because of subclinical endothelial damage due to the inflammatory systemic process itself, leading to kidney disease, cardiovascular disease up to hypertension in children with JIA.…”

Section: Introductionmentioning

confidence: 99%

Proteinuria and hematuria as early signs of renal involvement in juvenile idiopathic arthritis

Del Giudice,

Marcellino,

Hoxha

et al. 2024

Front. Pediatr.

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ObjectivesTo evaluate glomerular and tubular renal functions and analyze blood pressure in a cohort of pediatric patients with juvenile idiopathic arthritis (JIA).MethodsA total of 40 pediatric patients, 20 (50%) with JIA and 20 (50%) healthy control subjects, were studied, and performed the renal function on 24-h collection and the 24-h ambulatory blood pressure monitoring (ABPM). Moreover, we compared renal function and blood pressure trends between the groups of JIA patients with different disease activities.ResultsNo statistically significant differences were observed between patients with JIA and healthy children in terms of glomerular filtration rate (GFR), fractional excretion of sodium (FENa), tubular reabsorption of phosphate (TRP), and calcium-creatinine urine ratio (CaU/CrU). In contrast, we observed significantly higher values in JIA patients than in controls for the presence of hematuria (p < 0.0001) and proteinuria (p < 0.0001). Compared to the control group there were significantly higher values of hematuria and proteinuria/day in both groups of JIA patients with low disease activity (respectively, p = 0.0001 and p = 0.0002) and moderate disease activity (respectively p = 0.0001 and p = 0.0012). Systolic and diastolic dipping were significantly reduced in patients with JIA compared with healthy controls (p < 0.0001 and p < 0.0001, respectively).ConclusionsOur study showed that children with JIA, already in the early stages of the disease, have higher values of hematuria and proteinuria, which are early warning signs of nephropathy. Therefore, detailed screening of renal function and pressure monitoring in patients are necessary to monitor their evolution over time.

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Development and Implementation of the AIDA International Registry for Patients with Non-Infectious Scleritis

Cited by 12 publications

References 14 publications

Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome

Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome

Development and implementation of the AIDA international registry for patients with Schnitzler's syndrome

Proteinuria and hematuria as early signs of renal involvement in juvenile idiopathic arthritis

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