Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial.

OBJECTIVE: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative morbidity in children than tonsillectomy. We aimed to compare the efficacy of tonsillotomy to observation-only in children with PFAPA syndrome at a 3-month follow-up. METHODS: This was a randomized multicenter trial with sequential design. Participants were randomized into a tonsillotomy group and a control group that was only observed. The trial started in 1/2017 and was accomplished in 12/2021 with 16 patients (10 boys, six girls, the mean age 4.2 years). The symptoms were monitored with daily symptom diaries. RESULTS: After the 3-month follow-up, 7/8 patients (87.5%) in the tonsillotomy group and 2/8 (25%) patients in the control group were free from PFAPA symptoms (95% CI 13% to 87%; p = 0.0021). The mean number of days with fever was 2.6 (SD 3.7) in the tonsillotomy group and 8.0 (SD 6.5) days in the control group (n = 8) (p = 0.06). Mean number of fever days compatible with PFAPA syndrome was 0.8 (SD 1.4) in the tonsillotomy group and 6.5 (SD 6.0) in the control group (95%CI -10% to -1%; p = 0.007). Rescue tonsillectomy was needed for all patients in the control group and none of the patients in the tonsillotomy group. CONCLUSIONS: Tonsillotomy might be an effective treatment option for children with PFAPA syndrome. Further studies are needed to clarify the long-term efficacy of tonsillotomy for treating PFAPA. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:968-972, 2024.

Management of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis with Intracapsular Tonsillectomy.

OBJECTIVE: This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adenoidectomy (ITA). METHODS: We conducted a retrospective analysis of 2 children who were referred for an otolaryngology consultation between 2019 and 2022 for surgical treatment of PFAPA syndrome. Both patients had symptoms strongly suggestive of PFAPA and were at risk for total tonsillectomy (TT) complications. ITA was performed using a microdebrider. Both patients were followed up postoperatively to assess for symptomatic resolution and complications. RESULTS: Two children exhibited recurrent febrile episodes prior to ITA. The procedure was efficacious in both patients, with neither experiencing postoperative complications or recurring PFAPA symptoms for over 1 year after surgery. CONCLUSION: Our study reported on the use of ITA as a surgical treatment option for PFAPA. We showed that ITA eliminated febrile attacks and was safely performed without postoperative complications in 2 pediatric patients after 1-year follow-up. Future studies involving larger cohorts of PFAPA patients and lengthier follow-ups will need to be conducted to further evaluate ITA as a surgical option. Laryngoscope, 134:1967-1969, 2024.

Spinal Epidural Abscess and Strep Pharyngitis.

Spinal epidural abscesses (SEA) require prompt diagnosis to avoid devastating consequences. Here, we discuss the case of a healthy 20-year-old college student-with a recent diagnosis of strep pharyngitis-who presented with neck pain, fever, and a neurologic deficit-the most common symptoms of SEA. Magnetic resonance imaging revealed a T1-postcontrast, peripherally enhancing epidural collection from C3-T5 with associated cord compression and T3 osteomyelitis. The patient was treated with emergent skip hemilaminectomies for abscess evacuation. Surgical cultures grew Fusobacterium necrophorum, a highly unusual pathogen in SEA. It is an oral anaerobe that translocated through the mucosa in the setting of strep pharyngitis. We treated the patient with ceftriaxone for 6 weeks. The patient had a full neurologic recovery and remains without recurrence of infection 11 months postoperatively. Healthy patients without obvious risk factors may present with SEA, highlighting the need for atypical cases such as these to be brought to clinicians' attention.

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.

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.

The effect on the T-14 paediatric throat disorders outcome score of delaying adenotonsillectomy surgery due to COVID-19.

INTRODUCTION: Adenotonsillectomy is commonly performed for recurrent tonsillitis and obstructive sleep apnoea. These conditions may improve with age. The COVID-19 pandemic led to all UK elective surgery being suspended. This study aimed to determine whether delaying surgery had any effect on a patient's symptoms using the validated T-14 paediatric throat disorders outcome test. METHODS: Patients completed a T-14 questionnaire when the child was listed for surgery; this was repeated on the revised surgery date and a paired t test was used to compare the responses. RESULTS: Questionnaires were completed by 29 patients a mean of 6.4 months apart. There was a significant improvement in scores (p<0.02) for five domains: eating habits, visits to a doctor/A&E, antibiotics for less than 2 weeks, chronic infections and school missed due to sore throats. CONCLUSIONS: This study demonstrates that following delays resulting from the COVID-19 pandemic, paediatric patients experienced an improvement in some aspects of their quality of life while awaiting tonsillectomy and/or adenoidectomy. This was most apparent in quality-of-life measures relating to recurrent tonsillitis, namely visits to a doctor/A&E, antibiotics for less than 2 weeks, chronic infections and school missed due to sore throats. Patients may experience an improvement in some of their individual symptoms, in particular their infective symptoms, during an observation period.

Throat Symptoms Before and After Septal Surgery in Adults With Nasal Septum Deviation.

OBJECTIVE: To investigate subjective throat symptoms with the Qualities of Sore Throat Index (QuaSTI) in adults with nasal septum deviation. STUDY DESIGN: Prospective study. SETTING: Baskent University Ankara Hospital. METHODS: Group 1 included patients with septum deviation. Preoperative data were obtained through evaluation of subjective nasal obstruction with the NOSE (Nasal Obstruction Symptom Evaluation) and throat symptoms with the QuaSTI (group 1a). After 3 months, the same patient group was reevaluated with the NOSE and QuaSTI to obtain postoperative data (group 1b). Group 2 was formed of healthy volunteers with no nasal obstruction. RESULTS: The mean ± SD and median (range) values of the QuaSTI total score were 33.39 ± 29.50 and 18.5 (0-90) in group 1a, 7.49 ± 4.31 and 7 (0-18) in group 2, and 7.58 ± 9.24 and 4.50 (0-49) in group 1b (group 1a vs 2, P < .001; group 1a vs 1b, P < .0001). A significant difference was determined between groups 1a and 2 with respect to the QuaSTI sensory score (26.58 ± 23.54 and 18 [0-73] vs 6.51 ± 3.58 and 7 [0-15], P < .001, respectively) and between groups 1a and 1b for the QuaSTI functional score (6.03 ± 6.62 and 3 [0-20] vs 1.50 ± 2.47 and 0.50 [0-13], P = .004). No significant difference was found between groups 1a and 2 in terms of the QuaSTI emotional score (P = .126). CONCLUSION: Various persistent sensory and functional throat symptoms can be seen in patients experiencing nasal obstruction associated with septum deviation, and the majority of these symptoms recover after septal surgery.

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.

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean fever (FMF) causing treatment complexity. As the role of surgery in PFAPA syndrome is still controversial, in this paper, our aim is to present our results of tonsillectomy/adenotonsillectomy in the treatment of PFAPA syndrome. Archives of a tertiary care hospital were investigated for patients who underwent tonsillectomy or adenotonsillectomy due to PFAPA Syndrome between 2010 and 2020. 344 patients were found but only 281 of them were accessible. Through phone call interview and chart review methods, preoperative and postoperative the number and severity of the attacks and general satisfaction after the operation were recorded and analyzed. Also, patients with concomitant FMF were analyzed separately. A total of 281 patients were included in the study. There was no improvement in 10 (3.55%) patients. Eight (2.84%) patients showed mild improvement, 29 (10.32%) patients had moderate improvement and 234 (83.27%) patients had full recovery after tonsillectomy. There were 266 PFAPA patients without FMF. No improvement, mild improvement, moderate improvement, and full recovery in this patient group were 5 (1.9%), 6 (2.3%), 25 (9.4%) and 230 (86.5%), respectively. FMF was present in 5.33% (15/281) of the patients. In PFAPA + FMF group 5 patients had no improvement (33.3%), 2 had mild improvement (13.3%), 4 had moderate improvement (26.7%) and 4 had full recovery (26.7%). Benefit of tonsillectomy was significantly lower in the patients with concomitant FMF when compared to the patients who did not have FMF (p < 0.001). Age of diagnosis, age of operation, severity of the disease, type of operation, and gender were found to have no significant relationship with the benefit from surgery (p < 0.05). According to the findings of this study, tonsillectomy is an effective long-term treatment for PFAPA syndrome with success rate of 83.27%. Also, preoperatively FMF should be considered in these patients, which dramatically reduces surgical efficacy.

Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF).

OBJECTIVES: To describe a homogeneous group of patients with undifferentiated recurrent fevers followed-up in a tertiary referral center for systemic autoinflammatory diseases (SAIDs). METHODS: Patients with undifferentiated recurrent fevers seen at our Center from 2008 to 2021 and followed-up for at least one year were included in a retrospective study. Monogenic recurrent fevers, patients carrying variants of unknown origin and PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) syndrome were excluded. RESULTS: Fifty patients (34 male, 16 female) were included in the study. The median age at onset was 3 years, and the median follow-up was 3.3 years. At baseline, arthralgia (70%) and abdominal pain (65%) were the most frequent manifestations. NSAIDs or steroids on demand had a variable and transient effect. Tonsillectomy was ineffective in the 10 patients (20%) that underwent surgery. Forty-eight patients (96%) were treated with colchicine. A complete response (absence of fever) was achieved in 31 patients (64.6%). Nine patients (18%) showed a partial response, with a median reduction of fever episodes per year of 72%. Nine patients (16.7%) were considered resistant to colchicine. The presence of generalized lymphadenopathy and, to a lesser extent, exudative tonsillitis was associated with a lack of response to colchicine. CONCLUSIONS: We describe the largest series of patients with syndrome of undifferentiated recurrent fever (SURF) reported in the literature so far. SURF should be considered as a distinct clinical entity in the context of multifactorial autoinflammatory diseases.

Impact of the COVID-pandemic on the incidence of tonsil surgery and sore throat in Germany.

PURPOSE: To longitudinally evaluate the impact of the COVID-19-pandemic on the incidence of inpatient tonsil surgery and outpatient primary care of sore throat in Germany. METHODS: A retrospective interrupted time-series analysis was conducted. The national database of the Hospital Remuneration System was used to retrieve the number of operations performed between January 2019 and September 2021 including elective and non-elective cases with the exception of malign diseases. Three episodes were compared on a weekly basis: before, during, and after the first national lockdown (March 16-May 3, 2020). We also analysed the number of outpatient doctor contacts of sore throat patients in 2019 and 2020. RESULTS: Overall, 144,069 surgical cases were included in the analysis. The first lockdown resulted in an abrupt and significant decrease of all types of tonsil surgery (p < 0.01). The incidence of tonsillectomy decreased from 556 (before) to 111 (during) and 326 (after) cases per week (relative risk 0.24; 95% CI 0.19-0.30, and 0.82; 95% CI 0.73-0.92). After the lockdown, the incidence persisted on a lower level compared to 2019. The number of doctor contacts decreased from 2,967,322 in 2019 to 1,976,617 in 2020 (- 33.4%). CONCLUSIONS: The first lockdown was associated with a significant decrease of all types of tonsil surgery. A return to pre-pandemic surgical activity was not identified. The findings were accompanied by a significant decrease of outpatient doctor contacts of sore throat patients in primary care, particularly in the subgroup of children and adolescents. The impact of the second lockdown, starting in December 2020, was by far not comparable.

Comparison of the efficacy of supraglottic airway devices in low-risk adult patients: a network meta-analysis and systematic review.

Numerous supraglottic airway device (SADs) have been designed for adults; however, their relative efficacy, indicated by parameters such as adequacy of sealing, ease of application, and postinsertion complications, remains unclear. We conducted a systematic review and network meta-analysis to evaluate the efficacy of various SADs. We searched electronic databases for randomized controlled trials comparing at least two types of SADs published before December 2019. The primary outcomes were oropharyngeal leak pressure (OLP), risk of first-attempt insertion failure, and postoperative sore throat rate (POST). We included 108 studies (n = 10,645) comparing 17 types of SAD. The Proseal laryngeal mask airway (LMA), the I-gel supraglottic airway, the Supreme LMA, the Streamlined Liner of the Pharynx Airway, the SoftSeal, the Cobra Perilaryngeal Airway, the Air-Q, the Laryngeal Tube, the Laryngeal Tube Suction II, the Laryngeal Tube Suction Disposable, AuraGain, and Protector had significantly higher OLP (mean difference ranging from 3.98 to 9.18 cmH2O) compared with that of a classic LMA (C-LMA). The Protector exhibited the highest OLP and was ranked first. All SADs had a similar likelihood of first-attempt insertion failure and POST compared with the C-LMA. Our findings indicate that the Protector may be the best SAD because it has the highest OLP.Systematic review registration PROSPERO: CRD42017065273.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy.

OBJECTIVES/HYPOTHESIS: Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, but the role of adenoidectomy, as well as later tonsillar regrowth, is unclear. To find out if the volume of lymphoid tissue is pivotal to the efficacy, we analyzed the association between the relapse of the symptoms of PFAPA syndrome and regrowth of tonsillar tissue after tonsillectomy or adenotonsillectomy. STUDY DESIGN: Prospective cohort study of operated PFAPA pateints. METHODS: We invited all patients that had undergone tonsillectomy or adenotonsillectomy due to PFAPA syndrome at the Oulu University Hospital, Oulu, Finland, between the years 1990 and 2007, at the age of ≤12 years, to a follow-up visit, after an average period of 9.8 years after their diagnoses. Out of the 132 invited, 94 (71%) participated in the follow-up study. RESULTS: At the follow-up study visit, 5 (5%) of the 94 PFAPA syndrome cases experienced recurrent fevers. The regrowth of palatine tonsillar tissue was seen in four of them (80%) as compared to 19/89 (21%) of symptom-free patients (P = .006). Two of the patients with clear PFAPA relapse at the time of the study visit were reoperated with clear effect on the symptoms. At the time of the study visit, 59/63 (94%) of the patients who had undergone adenotonsillectomy and 30/31 of the patients (97%) who had undergone tonsillectomy earlier were free of fever flares (P = .99). CONCLUSION: Palatine tonsil regrowth was associated with PFAPA syndrome relapse after tonsillectomy. Reoperation might be a treatment option in these patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2149-E2152, 2021.

Second generation laryngeal mask airway during laparoscopic living liver donor hepatectomy: a randomized controlled trial.

The second-generation laryngeal mask airway (LMA) provides a higher sealing pressure than classical LMA and can insert the gastric drainage tube. We investigated the difference in respiratory variables according to the use of second-generation LMA and endotracheal tube (ETT) in laparoscopic living liver donor hepatectomy (LLDH). In this single-blind randomized controlled trial, intraoperative arterial carbon dioxide partial pressure at 2 h after the airway devices insertion (PaCO2_2h) was compared as a primary outcome. Participants were randomly assigned to the following groups: Group LMA (n = 45, used Protector LMA), or Group ETT (n = 43, used cuffed ETT). Intraoperative hemodynamic and respiratory variables including mean blood pressure (MBP), heart rate (HR), and peak inspiratory pressure (PIP) were compared. Postoperative sore throat, hoarseness, postoperative nausea and vomiting (PONV), and pulmonary aspiration were recorded. The PaCO2_2h were equally effective between two groups (mean difference: 0.99 mmHg, P = 0.003; 90% confidence limits: - 0.22, 2.19). The intraoperative change in MBP, HR, and PIP were differed over time between two groups (P < 0.001, P = 0.015, and P = 0.039, respectively). There were no differences of the incidence of postoperative complications at 24 h following LLDH (sore throat and hoarseness: P > 0.99, PONV: P > 0.99, and P = 0.65, respectively). No case showed pulmonary aspiration in both groups. Compared with endotracheal tube, second-generation LMA is equally efficient during LLDH. The second-generation LMA can be considered as the effective airway devices for securing airway in patients undergoing prolonged laparoscopic surgery. Trial Registration This study was registered at the Clinical Trial Registry of Korea ( https://cris.nih.go.kr . CRiS No. KCT0003711).

Lingual Tonsillectomy: Successful Treatment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome.

BACKGROUND: PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is diagnosed clinically. Adult-onset PFAPA syndrome is rare and often has a more diverse clinical presentation that its childhood counterpart. This is the first reported case of adult-onset PFAPA syndrome with complete response to lingual tonsillectomy. CASE SUMMARY: A 41-year-old man was evaluated for periodic fevers associated with uvulitis, cervical lymphadenitis, pharyngitis, and lower extremity rash. He had a variable response to steroids and was intolerant of colchicine. Laboratory workup revealed intermittent elevation of erythrocyte sedimentation rate and C-reactive protein level. Computed tomography neck and laryngoscopy confirmed adenoidal and lingual tonsillar hypertrophy. He underwent adenoidectomy and lingual tonsillectomy with resolution of symptoms. CONCLUSIONS: Hypertrophy of the remaining lymphoid structures within Waldeyer's ring may be associated with remote recurrence of PFAPA syndrome after tonsillectomy. Lingual tonsillectomy may be an alternative treatment strategy in select patients with PFAPA, prominent lingual hypertrophy, and incomplete response to steroids.

Pre- and postoperative quality of life and emotional/behavioural problems in children with PFAPA.

OBJECTIVE: This study aimed to evaluate the effects of surgical intervention on quality of life, emotional/behavioural problems and school absenteeism in children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA.) METHOD: A total of 56 children aged between 4 and 12 years, diagnosed with PFAPA and undergoing tonsillectomy with/without adenoidectomy at a tertiary hospital were included in the study. The Questionnaire for Quality of Life Assessment for Children and Adolescents Parent Form (Kid-KINDL) and the Strengths and Difficulties Questionnaires (SDQ) were used to evaluate quality of life and emotional/behavioural problems, respectively. The cases were assessed before and three months after surgery. RESULTS: The mean (SD) age of the children was 6.64 (3.03) years, and 58.9% (n = 33) were boys. The numbers of pre- and post-operative periodic fever episodes were 3.0 (1.7) and 0.6 (0.9) (p < 0.001), those for school absenteeism were 10.28 (5.53) and 2.85 (2.95) days (p < 0.001) and those for hospital presentations were 3.8 (2.5) and 1.1 (1.1) (p < 0.001), respectively, all of which were significantly lower in children with PFAPA at three months after the operation. The surgical procedures were effective in improving quality of life and emotional/behavioural problems, as evidenced by the significant differences between the pre- and post-treatment scores in outcome measures, including SDQ and Kid-KINDL (p < 0.001). CONCLUSIONS: This study demonstrated that the surgical approach exhibited positive effects in terms of improving quality of life and reducing emotional/behavioural problems and school absenteeism in children with PFAPA.

[CAUSES AND PECULIARITIES OF DEVELOPMENT OF CHRONIC ATROPHIC PHARYNGITIS].

The aim of the study was to identify the causes of the development of chronic atrophic pharyngitis in patients undergoing tonsillectomy in childhood or adolescence. The study included 145 patients (mean age 46.1 years), which were divided into two groups: the first group consisted of patients who visited a laryngologist during the acute illness - it included 32 patients (11 men, 21 women); The second group consisted of patients who had symptoms of chronic atrophic pharyngitis in recent years, and it included 113 patients (43 men, 70 women). The evaluation of the treatment results was carried out taking into account the anamnesis (subjective data) and the pharyngoscopic picture (objective data). Analysis of the study showed that 97 patients with symptoms of chronic atrophic pharyngitis in children and adolescents (5 to 16 years) underwent bilateral tonsillectomy and only in 48 patients atrophic processes in the throat were not associated with this operation.

Surgical outcomes and unique histological features of tonsils after tonsillectomy in adults with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

OBJECTIVES: Data on the adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome are scarce. European studies reported that unlike pediatric-onset PFAPA, tonsillectomy is ineffective for adult-onset PFAPA. The aims of this study were (1) to assess the response to tonsillectomy in a cohort of Japanese adult-onset PFAPA patients and (2) to evaluate the histologic appearance of tonsils in adult-onset PFAPA patients and to compare them with those of tonsils from age- and sex-matched controls with chronic tonsillitis. METHODS: In this retrospective cohort study, 5 adults with PFAPA and 15 controls who had undergone tonsillectomy were recruited. The size of the tonsil germinal centers was measured by hematoxylin and eosin staining, and the number and density of B and T lymphocytes in germinal centers were measured by immunohistochemistry, using CD3, CD4 and CD8 as T cell markers and CD20 as B cell marker. RESULTS: All patients had complete remission of the symptoms after surgery. PFAPA patients had significantly smaller germinal center areas than controls. The number and density of CD8+ cells in germinal centers were significantly lower in tonsils from PFAPA compared with controls. No differences were found between the two groups in CD3+, CD4+, and CD20+ cells. These results are compatible with the tonsillar features of pediatric-onset PFAPA. CONCLUSION: Our report demonstrates that tonsillectomy might be effective for adult-onset PFAPA and that tonsils of adult- and pediatric-onset PFAPA share the same histological features. These results suggest that the pathogenic mechanisms of adult- and pediatric-onset PFAPA are identical.

The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review.

OBJECTIVES: Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. However, an immunological reason for this effect remains unknown. This literature review summarizes the current knowledge regarding the immunological role of the tonsils in the PFAPA syndrome. METHODS: We searched PubMed, Medline, EMBASE and Cochrane for papers written in English dated from 1 January 1987 to 30 April 2019. The search included all studies reporting histological, immunological or microbiological workup of tonsil specimens from children aged 0-18 years with PFAPA. RESULTS: Thirteen articles reported histological, immunological or microbiological workup of tonsil specimens in children with PFAPA. The histology of tonsil specimens from children with PFAPA displayed chronic tonsillar inflammation with lymphoid hyperplasia. No uniform immunological pattern was identified, but some studies found fewer B-lymphocytes and smaller germinal centers in PFAPA compared to controls. A difference in tonsillar microbiota between PFAPA and controls was found in one study. CONCLUSION: A uniform immunological or microbiological pattern explaining the clinical effect of TE in children with PFAPA has not been revealed. Future targeted immunological studies of tonsils in PFAPA patients could possibly illuminate the understanding of the immunology in this disease.