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  • 11
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    Online Resource
    Preliminary data revealing efficacy of Streptococcus salivarius K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: A multicenter study from the AIDA Network PFAPA syndrome registry
    Frontiers Media SA ; 2023
    In:  Frontiers in Medicine Vol. 10 ( 2023-2-16)
    Details
    In: Frontiers in Medicine, Frontiers Media SA, Vol. 10 ( 2023-2-16)
    Abstract: To evaluate the potential role of Streptococcus salivarius 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 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months. Results The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p & lt; 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [ p & lt; 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p & lt; 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p & lt; 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis ( p & lt; 0.001), oral aphthae ( p & lt; 0.001) and cervical lymphadenopathy ( p & lt; 0.001) significantly decreased following SSK12. Conclusion SSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome.
    Type of Medium: Online Resource
    ISSN: 2296-858X
    URL: Article
    DOI: 10.3389/fmed.2023.1105605
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2023
    detail.hit.zdb_id: 2775999-4
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  • 12
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    Online Resource
    A patient-driven registry on Behçet’s disease: the AIDA for patients pilot project
    Frontiers Media SA ; 2023
    In:  Frontiers in Medicine Vol. 10 ( 2023-6-16)
    Details
    In: Frontiers in Medicine, Frontiers Media SA, Vol. 10 ( 2023-6-16)
    Abstract: 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. Results Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet’s Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0–30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1–50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range – 1.8–4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status ( p   & lt; 0.001), the presence of any major organ involvement ( p   & lt; 0.031), the presence of gastro-intestinal ( p   & lt; 0.001), neurological ( p  = 0.012) and musculoskeletal ( p  = 0.022) symptoms, recurrent fever ( p  = 0.002), and headache ( p   & lt; 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD ( F  = 14.519, OR 1.162 [CI 0.557–1.766], p   & lt; 0.001). Discussion Preliminary results from the AIDA for Patients BD registry were consistent with data available in the literature, confirming that PROs and PREs could be easily provided by the patient remotely to integrate physician-driven registries with complementary and reliable information.
    Type of Medium: Online Resource
    ISSN: 2296-858X
    URL: Article
    DOI: 10.3389/fmed.2023.1188021
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2023
    detail.hit.zdb_id: 2775999-4
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  • 13
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    Online Resource
    Development and Implementation of the AIDA International Registry for Patients With VEXAS Syndrome
    Frontiers Media SA ; 2022
    In:  Frontiers in Medicine Vol. 9 ( 2022-7-11)
    Details
    In: Frontiers in Medicine, Frontiers Media SA, Vol. 9 ( 2022-7-11)
    Abstract: 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. 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. Results To date (April 22 nd , 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. Conclusion This 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.
    Type of Medium: Online Resource
    ISSN: 2296-858X
    URL: Article
    DOI: 10.3389/fmed.2022.926500
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2775999-4
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  • 14
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    Online Resource
    The systemic score may identify life‐threatening evolution in Still's disease: data from the GIRRCS AOSD ‐study group and the AIDA Network Still's Disease Registry
    Wiley ; 2024
    In:  Arthritis & Rheumatology
    Details
    In: Arthritis & Rheumatology, Wiley
    Abstract: To evaluate the clinical usefulness of the systemic score in the prediction of life‐threatening evolution in Still's disease. To assess the clinical relevance of each component of the systemic score in predicting life‐threatening evolution and to derive patient subsets accordingly. Methods A multicenter, observational, prospective study was designed including patients included in the GIRRCS ( Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale ) AOSD‐study group and AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry. Patients were assessed if variables to derive the systemic score were available. The life‐threatening evolution was defined as mortality, whichever the clinical course, and/or macrophage activation syndrome (MAS), a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis. Results Totally 597 patients with Still's disease were assessed (age 36.6±17.3 years; male 44.4%). The systemic score, assessed as continuous variable, significantly predicted the life‐threatening evolution (OR: 1.24, 95%CI:1.07–1.42; p=0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life‐threatening evolution (OR: 3.36, 95%CI:1.81–6.25; p 〈 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR: 1.68, 95%CI:1.48–2.67; p=0.031) and lung disease (OR: 2.12, 95%CI:1.14–4.49; p=0.042) both significantly predicted life‐threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations. Conclusion The clinical utility of the systemic score was shown in identifying Still's disease at higher risk of life‐threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted. image
    Type of Medium: Online Resource
    ISSN: 2326-5191 , 2326-5205
    URL: Article
    DOI: 10.1002/art.42845
    RVK:
    XA 10000
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2024
    detail.hit.zdb_id: 2754614-7
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