Evidence map›Paper›PMID 41466290›Full record

ArticlePediatric rheumatology online journal2025

Symptom course of PFAPA syndrome.

Mathilde Labouret, Inès Elhani, Sébastien Cavelot, Glory Dingulu, Maurine Jouret, Ai Tien Nguyen, Adrien Subervie, Anne-Sophie Tavernier, Caroline Vinit, Véronique Hentgen

Abstract read
In one paragraph

Article in Pediatric rheumatology online journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Review
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Mathilde LabouretDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Inès ElhaniDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Sébastien CavelotDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Glory DinguluGeneral Pediatrics, Department of Infectious Disease and Internal Medicine, Reference Center for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), Robert Debré Mother-Child University Hospital, AP-HP, Paris, France.
Maurine JouretPediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mére Enfant, Reference Center for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), Hospices Civils de Lyon, Bron, France.
Ai Tien NguyenDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Adrien SubervieDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Anne-Sophie TavernierDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.
Caroline VinitGeneral Pediatrics, Department of Infectious Disease and Internal Medicine, Reference Center for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), Robert Debré Mother-Child University Hospital, AP-HP, Paris, France.
Véronique HentgenDepartment of General Pediatrics, Reference Center for Auto-inflammatory Diseases and Amyloidosis (CEREMAIA), Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France. vhentgen@ght78sud.fr.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPeriodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of autoinflammatory periodic fever in children. It is generally considered to be a self-limiting condition that resolves spontaneously over time.

objectivesTo evaluate age and delay to recovery of patients with PFAPA syndrome.

methodsWe retrospectively reviewed the medical records of patients diagnosed with PFAPA syndrome at the Versailles Hospital (Paris, France) and included in the Juvenile Inflammatory Rheumatism (JIR) cohort between 2016 and February 2023. Recovery was defined as the absence of any febrile PFAPA episode in the past year. Patients with either no reported febrile episode or insufficient information on fever status over the last 12 months were contacted by telephone.

results209 patients with PFAPA syndrome were included. Overall, 56 (27%) patients experienced resolution of periodic fever, 119 (57%) were still active and 34 (16%) patients were lost to follow-up. Among recovered patients, the median duration 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 patients with persistent fever, 10 patients were initially declared cured but relapsed.

conclusionIn patients whose fever resolved, most experienced their last febrile episode before adolescence. The identification of relapses after at least 12 months without a febrile episode raises questions about the definition of recovery.

Indexed as

FeverHereditary Autoinflammatory DiseasesLymphadenitisPharyngitisStomatitis, AphthousAdolescentChildChild, PreschoolFemaleHumansMaleRecurrenceRemission, SpontaneousRetrospective StudiesSyndromeOutcomePeriodic feverPFAPARecoveryRelapse

Identifiers

PMID41466290
PMCPMC12750835

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.