| Title: |
SYMPTOM COURSE OF PFAPA SYNDROME |
| Authors: |
Labouret, Mathilde; Elhani, Inès; Dingulu, Glory; Jouret, Maurine; Nguyen, Ai, Tien; Subervie, Adrien; Tavernier, Anne-Sophie; Vinit, Caroline; Hentgen, Véronique |
| Contributors: |
Hôpital Robert Debré; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital universitaire Robert Debré Reims (CHU Reims); Hôpital Jean Verdier AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Centre Hospitalier Sud Francilien - Centre Hospitalier d'Evry (CHSF); Hôpital Femme Mère Enfant CHU - HCL (HFME); Hospices Civils de Lyon (HCL); Centre Hospitalier de Versailles André Mignot (CHV); institutionnel : centre hospitalier de Versailles |
| Source: |
ISSN: 1546-0096 ; Pediatric Rheumatology ; https://hal.science/hal-05387473 ; Pediatric Rheumatology, In press ; https://link.springer.com/article/10.1186/s12969-025-01157-5. |
| Publisher Information: |
CCSD; BioMed Central |
| Publication Year: |
2025 |
| Collection: |
Hospices Civils de Lyon (HCL): HAL |
| Subject Terms: |
PFAPA periodic fever outcome recovery relapse; PFAPA; periodic fever; outcome; recovery; relapse; [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics; [SDV.IMM.II]Life Sciences [q-bio]/Immunology/Innate immunity |
| Description: |
International audience ; Background: Periodic fever, aphthous stoma99s, pharyngi9s and cervical adeni9s (PFAPA) syndrome is the most common cause of autoinflammatory periodic fever in children. It is generally considered to be a self-limi9ng condi9on that resolves spontaneously over 9me. Objec4ves: To evaluate age and delay to recovery of pa9ents with PFAPA syndrome. Methods: We retrospec9vely reviewed the medical records of pa9ents diagnosed with PFAPA syndrome at the Versailles Hospital (Paris, France) and included in the Juvenile Inflammatory Rheuma9sm (JIR) cohort between 2016 and February 2023. Recovery was defined as the absence of any febrile PFAPA episode in the past year. Pa9ents with either no reported febrile episode or insufficient informa9on on fever status over the last 12 months were contacted by telephone. Results: 209 pa9ents with PFAPA syndrome were included. Overall, 56 (27%) pa9ents experienced resolu9on of periodic fever, 119 (57%) were s9ll ac9ve and 34 (16%) pa9ents were lost to follow-up. Among recovered pa9ents, the median dura9on of symptoms was 5.43 years (Q1-Q3: 2.97-8.65) and the median age at last febrile episode was 8.34 years (Q1-Q3: 5.44-10.24). Of the 119 pa9ents with persistent fever, 10 pa9ents were ini9ally declared cured but relapsed. Conclusion: In pa9ents whose fever resolved, most experienced their last febrile episode before adolescence. The iden9fica9on of relapses aler at least 12 months without a febrile episode raises ques9ons about the defini9on of recovery. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Availability: |
https://hal.science/hal-05387473; https://hal.science/hal-05387473v1/document; https://hal.science/hal-05387473v1/file/Manuscript_evolutionaryprofileofPFAPAsyndrome_19sept25.pdf |
| Rights: |
https://about.hal.science/hal-authorisation-v1/ ; info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.90777E6F |
| Database: |
BASE |