Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever

Gözen, Emine Deniz; Yıldız, Mehmet; Kara, Sinem; Tevetoğlu, Fırat; Haşlak, Fatih; Adroviç, Amra; Şahin, Sezgin; Barut, Kenan; Ülkersoy, İpek; Gücüyener, Neslihan; Günalp, Aybüke; Yener, Murat; ADA, Mehmet; Kasapçopur, Özgür

doi:10.1007/s00296-022-05210-4

Cited by 5 publications

(4 citation statements)

References 45 publications

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“…[29][30][31] Tonsillectomy and adenoidectomy seem to be curative treatment options in most patients with PFAPA. 32 Although there were only 2 FDRs with PFAPA in the FMF group, we hypothesize that a significant amount of them were undiagnosed in the past, due to the newly growing and currently insufficient awareness of PFAPA among clinicians in our country.…”

Section: Discussionmentioning

confidence: 78%

Health conditions of first-degree relatives of children with familial Mediterranean fever

Yıldırım,

Haşlak,

Yıldız

et al. 2024

Turk J Pediatr

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Background. Given the strong genetic background of familial Mediterranean fever (FMF), the frequently reported co-existing diseases in children with FMF should also be investigated in other family members. Therefore, we aimed to examine the medical conditions of first-degree relatives (FDRs) of our pediatric patients with FMF in the present study. Methods. Chronic diseases of FDRs of pediatric 449 FMF, 147 juvenile idiopathic arthritis (JIA) patients and 93 healthy controls (HC) were questioned during their routine clinical visits for 9 consecutive months. Results. A total of 1975 FDRs of 449 FMF, 690 FDRs of 147 JIA patients, and 406 FDRs of 93 HC were included into the study. The most common medical conditions were non-atopic asthma (n=71, 3.6%), type 2 DM (n=14, 2%), and tonsillectomy history (n=12, 2.95%) in the FMF, JIA, and HC groups, respectively. Atopic diseases (FMF vs. JIA: p=0.013; FMF vs. HC: p=0.014), rheumatic diseases (FMF vs. JIA: p=0.030; FMF vs. HC: p=0.017), and surgical histories (FMF vs. JIA: p<0.01; FMF vs. HC: p=0.026), including adenoidectomy, tonsillectomy, and appendectomy, were significantly more common in the FMF group than in other groups. Conclusions. Our novel findings may contribute to understanding the hereditary burden of co-existing diseases in children with FMF and encourage further studies involving genetic screenings.

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Section: Discussionmentioning

confidence: 78%

Health conditions of first-degree relatives of children with familial Mediterranean fever

Yıldırım,

Haşlak,

Yıldız

et al. 2024

Turk J Pediatr

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“…Patients with late age of onset and headache were more likely to have a full response to tonsillectomy (lack of arthralgia was also a predictive factor in the univariate analysis but was not significant in a multivariable analysis). Another study of Turkish patients with PFAPA showed that those with concomitant FMF and PFAPA were significantly less likely to respond to tonsillectomy than those with PFAPA alone [41]. These differences in tonsillectomy efficacy based on symptoms suggest that subgroups of PFAPA may have differences in their pathogenesis and involvement of the tonsils.…”

Section: New Data On Treatment Strategiesmentioning

confidence: 99%

What is PFAPA syndrome? Genetic clues about the pathogenesis

Manthiram

2023

Current Opinion in Rheumatology

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Purpose of review Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in childhood. Recent studies report genetic susceptibility variants for PFAPA syndrome and the efficacy of tonsillectomy in a broader cohort of patients with recurrent stereotypical fever. In this review, we highlight the findings of these studies and what they may reveal about the pathogenesis of PFAPA. Recent findings Newly identified genetic susceptibility loci for PFAPA suggest that it is a complex genetic disorder linked to Behçet's disease and recurrent aphthous ulcers. Patients who have PFAPA with some features of Behçet's disease have been reported. Moreover, the efficacy of tonsillectomy has now been described in patients who do not meet the full diagnostic criteria for PFAPA, although the immunologic profile in the tonsils is different from those with PFAPA. Factors that predict response to tonsillectomy are also reported. Summary These findings highlight the heterogeneous phenotypes that may be related to PFAPA due to common genetic susceptibility or response to therapy. These relationships raise questions about how to define PFAPA and highlight the importance of understanding of the genetic architecture of PFAPA and related diseases.

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“…Single doses of corticosteroids have been shown to effectively terminate fever episodes in most patients with PFAPA, but use of corticosteroids is often associated with shortening of the fever-free intervals [ 6 ]. Tonsillectomy has been suggested to expedite recovery in most patients [ 7 – 9 ], but remains debated as the procedure is associated with some risks, certainty of evidence is moderate and PFAPA most often resolves spontaneously eventually [ 10 ]. Suffering from recurrent fevers for years may nevertheless have consequences for both the patients and their families.…”

Section: Introductionmentioning

confidence: 99%

Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children

Rydenman

Sparud-Lundin

Karlsson-Bengtsson

et al. 2023

Orphanet J Rare Dis

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Background Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder that primarily affects young children, and typically gives rise to fever episodes that recur monthly for several years. This study investigated the impact of PFAPA syndrome on the families of affected children, the health-related quality of life (HRQOL) of children with the syndrome, and how these factors were influenced by tonsillectomy. Methods This prospective cohort study included 24 children with typical PFAPA syndrome that were referred for tonsillectomy, of whom 20 underwent the procedure. The control group consisted of randomly selected children from the general population. Family impact and HRQOL were measured using the standardized, validated questionnaires Pediatric Quality of Life Inventory™ (PedsQL™) Family Impact Module (FIM) and PedsQL™ 4.0 Generic Core Scales (GCS). Parents to children with PFAPA completed the questionnaires before and 6 months after their child underwent tonsillectomy, and HRQOL was measured both between and during PFAPA episodes. The Wilcoxon signed-rank test was used to compare data before and after tonsillectomy in the patient group, while the Mann–Whitney test was used for comparison of the patient and control groups. Results Before tonsillectomy, children with PFAPA had significantly lower scores than the control group on the PedsQL™ FIM and the PedsQL™ 4.0 GCS during fever episodes. After tonsillectomy, all patients improved with diminished febrile episodes, which resulted in significantly higher scores regarding both family impact and HRQOL at the time of follow-up. HRQOL of in children with PFAPA improved after tonsillectomy even when compared to afebrile intervals before the procedure. The differences between PFAPA patients and controls were eliminated after tonsillectomy. Conclusion PFAPA syndrome has a profound negative impact on the families of affected children. Tonsillectomy that leads to cessation or reduction of fever episodes eases the impact of the disease on the family. HRQOL in children with PFAPA is low during febrile episodes and similar to healthy controls in between episodes. The improvement of HRQOL in patients with PFAPA after tonsillectomy compared to the afebrile intervals before tonsillectomy highlights that the constantly recurring fevers may affect the children’s well-being even between fever episodes.

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Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever

Cited by 5 publications

References 45 publications

Health conditions of first-degree relatives of children with familial Mediterranean fever

Health conditions of first-degree relatives of children with familial Mediterranean fever

What is PFAPA syndrome? Genetic clues about the pathogenesis

Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children

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