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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

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.