ReviewMediators of inflammation2015
The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research.
Review in Mediators of inflammation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled 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.
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.
Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 34 citations in OpenAlex.
- Surgical versus medical management of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis): a systematic review and network meta-analysis.Pediatric rheumatology online journal · 2026Pooled it
- Vaccination coverage, immune responses, and clinical characteristics in children with PFAPA syndrome: a monocentric prospective study.Pediatric rheumatology online journal · 2025Article
- Quality of Life and Psychological Functioning in Children with PFAPA Syndrome.Pediatric reports · 2025Article
- Adult-Onset PFAPA Syndrome: Successful Management with Colchicine.The American journal of case reports · 2024Article
- Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients.Pediatric rheumatology online journal · 2023Article
- PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study.Pediatric rheumatology online journal · 2022Article
- PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort.International journal of general medicine · 2022Article
- New insights on multigenic autoinflammatory diseases.Therapeutic advances in musculoskeletal disease · 2022Review
- Oscillations in a white blood cell production model with multiple differentiation stages.Journal of mathematical biology · 2020Article
- Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome persisting to adulthood - an example of a diagnostic and therapeutic challenge.Reumatologia · 2019Article
- High Prevalence of Autoantibodies Against Monomeric C Reactive Protein (CRP) in Children with PFAPA Syndrome.Journal of medical biochemistry · 2018Article
- Pregnancy complicated with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome: a case report.BMC pregnancy and childbirth · 2018Article
- A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome.Clinical rheumatology · 2017Article
- Procalcitonin and C-reactive protein-based decision tree model for distinguishing PFAPA flares from acute infections.Bosnian journal of basic medical sciences · 2016Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPFAPA syndrome is a chronic disease that is characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Knowledge regarding the etiology of PFAPA is limited.
objectivesTo provide up-to-date information considering etiology of PFAPA syndrome, by summarizing what has been explored and established in this area so far. MATERIALS AND
methodsPubMed, Web of Science, and Scopus databases were searched for pertinent reports. Eventually 19 articles were selected. The results were classified into categories regarding three areas of interest: familial occurrence, genetic basis, and immunological mechanisms of PFAPA.
resultsRecent findings suggest that there is a familial tendency to PFAPA but the level of evidence does not warrant definite conclusions. The absence of a clear monogenic trait indicates a heterogenous, polygenic, or complex inheritance of PFAPA syndrome. As two mutations with a possible functional effect on the inflammasomes (MEFV E148Q and NLRP3 Q703K) have been found in several PFAPA cohorts, the role of inflammasome-related genes in PFAPA pathogenesis cannot be excluded. Immunological mechanisms of PFAPA involve an abnormal, IL-1β dependent innate immune response to an environmental trigger, which leads to Th1-driven inflammation expressed by recruitment of T-cells to the periphery.
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Registered trials
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.