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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 Publisher



La Torre F1 ; Sota J2 ; Insalaco A3 ; Conti G4 ; Del Giudice E5 ; Lubrano R5 ; Breda L6 ; Maggio MC7 ; Civino A8 ; Mastrorilli V1 ; Loconte R1 ; Natale MF3 ; Celani C3 ; Romeo M4 Show All Authors
Authors
  1. La Torre F1
  2. Sota J2
  3. Insalaco A3
  4. Conti G4
  5. Del Giudice E5
  6. Lubrano R5
  7. Breda L6
  8. Maggio MC7
  9. Civino A8
  10. Mastrorilli V1
  11. Loconte R1
  12. Natale MF3
  13. Celani C3
  14. Romeo M4
  15. Patroniti S4
  16. Gentile C6
  17. Vitale A2
  18. Caggiano V2
  19. Gaggiano C2
  20. Diomeda F8
  21. Cattalini M9
  22. Lopalco G10
  23. Emmi G11, 12
  24. Parronchi P11
  25. Gentileschi S2
  26. Cardinale F1
  27. Aragona E13
  28. Shahram F14
  29. Marino A15
  30. Barone P16
  31. Moscheo C17
  32. Ozkiziltas B18
  33. Carubbi F19
  34. Alahmed O20
  35. Iezzi L21
  36. Ogunjimi B22, 23
  37. Mauro A24
  38. Tarsia M25
  39. Mahmoud AAMA26
  40. Giardini HAM27
  41. Sfikakis PP28
  42. Laskari K28
  43. Wiesikszewczyk E29
  44. Hernandezrodriguez J30
  45. Frediani B2
  46. Gomezcaverzaschi V30
  47. Tufan A18
  48. Almaghlouth IA31, 32
  49. Balistreri A33
  50. Ragab G26, 34
  51. Fabiani C35
  52. Cantarini L2
  53. Rigante D21, 36
Show Affiliations
Authors Affiliations
  1. 1. Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy
  2. 2. Division of Rheumatology, Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behcet’s Disease Clinic, University of Siena, Siena, Italy
  3. 3. Ospedale Pediatrico Bambino Gesu, IRCCS (European Reference Network for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases Center), Rome, Italy
  4. 4. Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria G. Martino, Messina, Italy
  5. 5. Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Latina, Italy
  6. 6. Pediatric Rheumatology Unit, S.S. Annunziata Hospital, Chieti, Italy
  7. 7. Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) “G. D’Alessandro�, University of Palermo, Palermo, Italy
  8. 8. Pediatric Rheumatology and Immunology Unit, Vito Fazzi Hospital, Lecce, Italy
  9. 9. Pediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia, Italy
  10. 10. Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
  11. 11. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy
  12. 12. Centre for Inflammatory Diseases, Department of Medicine, Monash Medical Centre, Monash University, Clayton, VIC, Australia
  13. 13. Division of Gastroenterology, Ospedali Riuniti Villa Sofia-Vincenzo Cervello, Palermo, Italy
  14. 14. Behcet’s Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  15. 15. Unit of Pediatric Rheumatology, ASST Gaetano Pini-CTO, Milan, Italy
  16. 16. Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
  17. 17. Pediatric Unit, Azienda Ospedaliero-Universitaria Policlinico “Rodolico-San Marco�, Catania, Italy
  18. 18. Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey
  19. 19. Department of Life, Health & Environmental Sciences, Internal Medicine and Nephrology Unit, Department of Medicine, University of L’Aquila and ASL Avezzano-Sulmona-L’Aquila, San Salvatore Hospital, L’Aquila, Italy
  20. 20. Pediatric Rheumatology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  21. 21. Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
  22. 22. Department of Pediatrics, Antwerp University Hospital, Antwerp, Belgium
  23. 23. Center for Health Economics Research and Modelling Infectious Diseases (CHERMID), Vaccine and Infectious Disease Institute, University of Antwerp, Wilrijk, Belgium
  24. 24. Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy
  25. 25. Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy
  26. 26. Rheumatology and Clinical Immunology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Giza, Egypt
  27. 27. Rheumatology Division, Hospital das Clinicas (HCFMUSP), Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil
  28. 28. Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  29. 29. Department of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, Warsaw, Poland
  30. 30. Vasculitis Research Unit, Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, August Pi i Sunyer Biomedical Research Institute (IDIBAPS), University of Barcelona, Barcelona, Spain
  31. 31. Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  32. 32. College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
  33. 33. Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy
  34. 34. Faculty of Medicine, Newgiza University, 6th of October City, Egypt
  35. 35. Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy
  36. 36. Rare Diseases and Periodic Fevers Research Centre, Universita Cattolica del Sacro Cuore, Rome, Italy

Source: Frontiers in Medicine Published:2023


Abstract

Objective: 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 < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p < 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 < 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 < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p < 0.001), oral aphthae (p < 0.001) and cervical lymphadenopathy (p < 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. Copyright © 2023 La Torre, Sota, Insalaco, Conti, Del Giudice, Lubrano, Breda, Maggio, Civino, Mastrorilli, Loconte, Natale, Celani, Romeo, Patroniti, Gentile, Vitale, Caggiano, Gaggiano, Diomeda, Cattalini, Lopalco, Emmi, Parronchi, Gentileschi, Cardinale, Aragona, Shahram, Marino, Barone, Moscheo, Ozkiziltas, Carubbi, Alahmed, Iezzi, Ogunjimi, Mauro, Tarsia, Mahmoud, Giardini, Sfikakis, Laskari, Wiesik-Szewczyk, Hernandez-Rodriguez, Frediani, Gomez-Caverzaschi, Tufan, Almaghlouth, Balistreri, Ragab, Fabiani, Cantarini and Rigante.
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