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Development and Implementation of the Aida International Registry for Patients With Vexas Syndrome Publisher



Vitale A1 ; Caggiano V1 ; Della Casa F2 ; Hernandezrodriguez J3 ; Frassi M4 ; Monti S5, 6 ; Tufan A7 ; Telesca S1 ; Conticini E1 ; Ragab G8, 9 ; Lopalco G10 ; Almaghlouth I11, 12 ; Pereira RMR13 ; Yildirim D7 Show All Authors
Authors
  1. Vitale A1
  2. Caggiano V1
  3. Della Casa F2
  4. Hernandezrodriguez J3
  5. Frassi M4
  6. Monti S5, 6
  7. Tufan A7
  8. Telesca S1
  9. Conticini E1
  10. Ragab G8, 9
  11. Lopalco G10
  12. Almaghlouth I11, 12
  13. Pereira RMR13
  14. Yildirim D7
  15. Cattalini M14
  16. Marino A15
  17. Giani T16
  18. La Torre F17
  19. Ruscitti P18
  20. Aragona E19
  21. Wiesikszewczyk E20
  22. Del Giudice E21
  23. Sfikakis PP22
  24. Govoni M23
  25. Emmi G24
  26. Maggio MC25
  27. Giacomelli R26
  28. Ciccia F27
  29. Conti G28
  30. Aitidir D29
  31. Lomater C30
  32. Sabato V31
  33. Piga M32
  34. Sahin A33
  35. Oprisbelinski D34
  36. Ionescu R34
  37. Bartoloni E35
  38. Franceschini F4
  39. Parronchi P24
  40. De Paulis A2, 36
  41. Espinosa G3
  42. Maier A37
  43. Sebastiani GD38
  44. Insalaco A39
  45. Shahram F40
  46. Sfriso P41
  47. Minoia F42
  48. Alessio M43
  49. Makowska J44
  50. Hatemi G45
  51. Akkoc N46
  52. Li Gobbi F47
  53. Gidaro A48
  54. Olivieri AN49
  55. Almayouf SM50
  56. Erten S51
  57. Gentileschi S52
  58. Vasi I7
  59. Tarsia M1
  60. Mahmoud AAMA8
  61. Frediani B52
  62. Fares Alzahrani M53
  63. Laymouna AH8
  64. Ricci F14
  65. Cardinale F17
  66. Jahnzrozyk K20
  67. Tosi GM54
  68. Crisafulli F4
  69. Balistreri A55
  70. Dagostin MA13
  71. Ghanema M8
  72. Gaggiano C1
  73. Sota J1
  74. Di Cola I18
  75. Fabiani C54
  76. Giardini HAM13
  77. Renieri A56, 57, 58
  78. Fabbiani A56, 57, 58
  79. Carrer A56, 57, 58
  80. Bocchia M59
  81. Caroni F59
  82. Rigante D60, 61
  83. Cantarini L1
Show Affiliations
Authors Affiliations
  1. 1. Department of Medical Sciences, Surgery, and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behcet's Disease Clinic, University of Siena, Siena, Italy
  2. 2. Section of Clinical Immunology, Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy
  3. 3. Vasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Institut d'Investigacions Biomediques August Pi i Sunyer, University of Barcelona, Barcelona, Spain
  4. 4. Rheumatology and Clinical Immunology Unit, Department of Clinical and Experimental Sciences, ASST Spedali Civili, University of Brescia, Brescia, Italy
  5. 5. Rheumatology Department, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy
  6. 6. Department of Experimental Medicine, University of Pavia, Pavia, Italy
  7. 7. Department of Internal Medicine and Rheumatology, Gazi University, Ankara, Turkey
  8. 8. Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt
  9. 9. Faculty of Medicine, Newgiza University (NGU), Giza, Egypt
  10. 10. Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
  11. 11. Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  12. 12. College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  13. 13. Rheumatology Division, Faculdade de Medicina, Hospital das Clinicas HCFMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil
  14. 14. Pediatric Clinic, Spedali Civili di Brescia, University of Brescia, Brescia, Italy
  15. 15. Unit of Pediatric Rheumatology, ASST Gaetano Pini-CTO, Milan, Italy
  16. 16. Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, ASST G. Pini-CTO, University of Milan, Milan, Italy
  17. 17. Department of Pediatrics, Pediatric Rheumatology Center, Ospedale “Giovanni XXIII�, Azienda Ospedaliera-Universitaria Consorziale Policlinico, Bari, Italy
  18. 18. Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy
  19. 19. Division of Gastroenterology, Ospedali Riuniti Villa Sofia-Vincenzo Cervello, Palermo, Italy
  20. 20. Department of Internal Medicine, Pulmonology, Allergy and, Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, Warsaw, Poland
  21. 21. Department of Maternal Infantile and Urological Sciences, Polo Pontino, Sapienza University of Rome, Rome, Italy
  22. 22. Joint Academic Rheumatology Program, 1st Department of Propedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
  23. 23. Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna–Ferrara, University of Ferrara, Ferrara, Italy
  24. 24. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
  25. 25. University Department Pro.Sa.M.I. “G. D'Alessandro�, University of Palermo, Palermo, Italy
  26. 26. Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, Universita Campus Bio-Medico di Roma, Selcetta, Italy
  27. 27. Department of Precision Medicine, Universita Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy
  28. 28. Pediatric Nephrology and Rheumatology Unit, AOU G Martino, Messina, Italy
  29. 29. Research Laboratory, Biodiversity, Biotechnology, Environment and Sustainable Development, Faculty of Sciences, M'Hamed Bougara University, Boumerdes, Algeria
  30. 30. Unita Operativa (UO) Reumatologia, AO Ordine Mauriziano, Turin, Italy
  31. 31. Department of Immunology, Allergology, and Rheumatology, Antwerp University Hospital, University of Antwerp, Antwerp, Belgium
  32. 32. Rheumatology Unit, Department of Medical Sciences, University and Azienda Ospedaliera-Universitaria of Cagliari, Cagliari, Italy
  33. 33. Division of Rheumatology, Department of Internal Medicine, Sivas Cumhuriyet University, Sivas, Turkey
  34. 34. Department of Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
  35. 35. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy
  36. 36. Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy
  37. 37. Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy
  38. 38. Unita Operativa Complessa (U.O.C.) Reumatologia, Ospedale San Camillo-Forlanini, Rome, Italy
  39. 39. Division of Rheumatology, Ospedale Pediatrico Bambino Gesu, IRCCS (European Reference Network for Immunodeficiency, Autoinflammatory and Autoimmune Diseases Center), Rome, Italy
  40. 40. Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  41. 41. Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, Italy
  42. 42. Pediatric Rheumatology, Fondazione IRCCS (Istituto di ricovero e cura a carattere scientifico) Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
  43. 43. Pediatric Rheumatology Unit, Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy
  44. 44. Department of Rheumatology, Medical University of Lodz, Lodz, Poland
  45. 45. Division of Rheumatology, Department of Internal Medicine, Behcet's Disease Research Center, Istanbul University–Cerrahpasa, Istanbul, Turkey
  46. 46. Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey
  47. 47. Rheumatology Unit, San Giovanni di Dio Hospital, Firenze, Italy
  48. 48. Department of Biomedical and Clinical Sciences 'Luigi Sacco', University of Milan, Milan, Italy
  49. 49. Department of Woman, Child and of General and Specialized Surgery, University of Campania “Luigi Vanvitelli�, Naples, Italy
  50. 50. King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
  51. 51. Department of Rheumatology, Ankara City Hospital, Ankara, Turkey
  52. 52. Unit of Rheumatology, Azienda Ospedaliero-Universitaria Senese, Siena, Italy
  53. 53. Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  54. 54. Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy
  55. 55. Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy
  56. 56. Medical Genetics, Department of Medical Biotechnologies, University of Siena, Siena, Italy
  57. 57. Department of Medical Biotechnologies, Med Biotech Hub and Competence Center, University of Siena, Siena, Italy
  58. 58. Genetica Medica, Azienda Ospedaliero-Universitaria Senese, Siena, Italy
  59. 59. Unit of Hematology, Azienda Ospedaliera Universitaria Senese, University of Siena, Siena, Italy
  60. 60. Department of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  61. 61. Rare Diseases and Periodic Fevers Research Centre, Universita Cattolica del Sacro Cuore, Rome, Italy

Source: Frontiers in Medicine Published:2022


Abstract

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. 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 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. 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. Copyright © 2022 Vitale, Caggiano, Della Casa, Hernandez-Rodriguez, Frassi, Monti, Tufan, Telesca, Conticini, Ragab, Lopalco, Almaghlouth, Pereira, Yildirim, Cattalini, Marino, Giani, La Torre, Ruscitti, Aragona, Wiesik-Szewczyk, Del Giudice, Sfikakis, Govoni, Emmi, Maggio, Giacomelli, Ciccia, Conti, Ait-Idir, Lomater, Sabato, Piga, Sahin, Opris-Belinski, Ionescu, Bartoloni, Franceschini, Parronchi, de Paulis, Espinosa, Maier, Sebastiani, Insalaco, Shahram, Sfriso, Minoia, Alessio, Makowska, Hatemi, Akkoc, Li Gobbi, Gidaro, Olivieri, Al-Mayouf, Erten, Gentileschi, Vasi, Tarsia, Mahmoud, Frediani, Fares Alzahrani, Laymouna, Ricci, Cardinale, Jahnz-Rozyk, Tosi, Crisafulli, Balistreri, Dagostin, Ghanema, Gaggiano, Sota, Di Cola, Fabiani, Giardini, Renieri, Fabbiani, Carrer, Bocchia, Caroni, Rigante and Cantarini.
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