Pfapa-syndrome in children: should remove or save the tonsils?

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Pfapa-syndrome in children: should remove or save the tonsils?

S. Levytska

Higher State Educational Establishment of Ukraine «Bukovinian State Medical University»

(Chernivtsi Ukraine)

Summary

pfapa syndrome tonsillectomy

The etiology of PFAPA syndrome is still unknown and due to that there is no specific laboratory test and no specific treatment. Some cases of successful treatment of patients with PFAPA through tonsillectomy were described in literature, but their level of evidence is not enough to consider the PFAPA-syndrome as indication for tonsillectomy.

Material and methods. For determination of the frequency of PFAPA-syndrome in children, who were aimed at the routine tonsillectomy, and the efficiency of the tonsillectomy in children with PFAPA-syndrome the analysis of the results of the examination and treatment of the 216 children was performed. The rheumo tests and the identification the beta-hemolytic streptococcus in the pharynx were performed to estimate the bacterial background of the tonsillitis. PFAPA-syndrome was diagnosed according to the Thomas criteria. For distinguish the PFAPA-episodes from the others cases of the throat infection we constructed and tested the special questionnaire.

The efficacy of the tonsillectomy was estimated by counting the episodes of upper respiratory tract infection, the amount of courses of antibiotic therapy as well as the amount of days of not attending the schools because of the throat infection during the year after tonsillectomy.

Results

PFAPA-syndrome according to the criteria Thomas was diagnosed in 6 children (3,3%). The average age ofpatients with PFAPA was 4 years, the average period between febrile episodes - 28 days.

The two groups were formed according to the results of examination: the first group of the 23 children with bacterial origin of tonsillitis (with presence the hemolytic streptococcus in the oropharynx as well as the high titer of antistreptolysin O); and the second group of the 6 children with symptoms that were assessed as PFAPA-syndrome.

The testing of the constructed questionnaire showed that the score in children of the first group rangedfrom 8 to 20 points and the score of the children with the PFAPA syndrome rangedfrom 22 to 32 points.

After the year after surgery the four and more episodes of throat infection were revealed in 4 children (17,4%) of the first group. The average amount of courses of antibiotic treatment per year has decreased from 6,4 to 1,8.

The complete disappearance of the fever episodes in a year after surgery was established in five cases of PFAPA- syndrome. The amount of episodes of upper respiratory tract infections in this group decreasedfrom 9,5 to 0,7; the amount of courses of antibiotic treatment - from 10,3 to 1,4; the amount of days not-attending the schools because of throat infection - from 68,4 to 14,8.

Conclusion. The frequency of the PFAPA-syndrome among tonsillectomy children was 3,3%. The constructed questionnaire improves the differential diagnosis process of the episodes of throat infection in children. Thus, the total number ofpoints less than 10 may indicate the bacterial origin of the chronic tonsillitis, but the total number of points above 24 points indicates the high probability of PFAPA-syndrome. Tonsillectomy is an effective treatment for children with PFAPA syndrome.

Key words: PFAPA; tonsillectomy; children.

Резюме

РТАРА-СИНДРОМ У ДГГЕЙ: ВИДАЛЯТИ ЧИ ЗБЕРІГАТИ МИГДАЛИКИ?

С.А. Левицька

Вищий Державний Навчальний

pfapa syndrome tonsillectomy

PFAPA-СИНДРОМ У ДЕТЕЙ: УДАЛЯТЬ И ХРАНИТЬ МИНДАЛИНЫ?

С.А. Левицкая

Высшее Государственное учебное заведение Украины «Буковинский государственный медицинский университет»

(г. Черновцы, Украина)

Этиология PFAPA-синдрома все еще остается неизвестной и поэтому до сих пор не сущетсвует специфического лабораторного теста или специфического лечения заболевания.

В литературе описаны случаи успешного лечения пациентов с PFAPA-синдромом посредством проведения тонзиллэктомии, однако, уровень их доказательности недостаточен для того, чтобы PFAPA-синдром можно было считать показаним к проведению тонзиллэктомии.

Материал и методы. Для определения частоты PFAPA-синдрома среди детей, направленных на проведение плановой тонзиллэктомии, а также для определения эффективности тонзиллэктомии при лечении детей с PFAPA-синдромом проведен анализ результатов обследования и лечения 216 детей. Для оценки бактериальной составляющей в развитии тонзиллита проводили определение ревмопроб и идентификацию бета-гемолитического стрептококка в глотке. PFAPA-синдром диагностировали на основании критериев Томас. Для дифференциации PFAPA-епизодов и других случаев заболеваний горла разработана и апробирована специальная анкета.

Эффективность тонзиллэктомии определяли с помощью подсчета количества эпизодов инфекции верхних дыхательных путей, количества курсов антибактериальной терапии и количества дней, на протяжении которых ребенок не посещал школу/ детский сад из-за заболевания горла на протяжении года после тонзиллэктомии.

Результаты. Согласно критериям Томас PFAPA- синдром диагностирован у 6 детей (3,3%). Средний возраст пациентов с PFAPA-синдромом - 4 года. Средний период между фебрильными эпизодами - 28 дней. Согласно результатам обследования сформировано две группы: в первую группу вошли 23 ребенка с бактериальным происхождением тонзиллита (идентифицированы гемолитический стрептококк в ротоглотке и повышенный титр антистрепто- лизина О); во вторую - 6 детей с симптомами, определенными как PFAPA-синдром. Апробация разработанной анкеты показала, что сумма баллов у детей первой группы колебалась от 8 до 20, у де¬тей с PFAPA-синдромом - от 22 до 32 баллов. Че¬рез год после операции четыре и больше эпизодов инфекции горла выявлено у 4 детей (17,4%) пер¬вой группы. Среднее количество курсов антиби- отикотерапии за год снизилось с 6,4 до 1,8. По¬лное исчезновение фебрильных эпизодов через год после операции зафиксировано в пяти случаях PFAPA-синдрома. У одного ребенка сохранились эпизоды фебрильной температуры, но интервал между эпизодами вырос с 26 до 90 дней. Количе¬ство случаев инфекций верхних дыхательных пу¬тей в этой группе уменьшилось с 9,5 до 0,7; коли¬чество курсов антибактериальной терапии - с 10,3 до 1,4; количество дней, на протяжении которых ребенок не посещал школу/детский сад из-за бо¬лезней горла - с 68,4 до 14,8.

Заключение. Частота PFAPA-синдрома среди тонзиллэктомированных детей составила 3,3%. Разработанная анкета улучшает процесс дифференциальной диагностики эпизодов инфекции горла у детей. Так, общая сумма баллов меньше 10 может указывать на бактериальное происхождение хронического тонзиллита, тогда как сумма баллов больше 24 указывает на высокую вероятность PFAPA-синдрома. Тонзиллэктомия оказалась эффективным методом лечения детей с PFAPA-синдромом.

The first analyses of the twelve clinical cases that are characterized by the combination of four symptoms:periodic fever (PF), aphthous stomatitis (A), pharyngitis or tonsilopharyngitis (P), inflammation of cervical lymph nodes (A - adenitis) were published by the group of French researchers in 1987 [1]. As for the first letters the new disease was called PFAPA-syndrome.

Since then, new data about the diagnosis and treatment of the patients with PFAPA has repeatedly appeared in the scientific literature [2, 3].

In spite of the long-term history of the investigation of PFAPA syndrome the etiology of this disease is still unknown and almost always the diagnosis of PFAPA syndrome is the diagnosis of exclusion [4]. There is no specific laboratory test to diagnose PFAPA as well as specific treatment.

The peculiarities of the PFAPA syndrome are the poor effect of the antipyretics and good effect of the steroids in decreasing the temperature [5]. Some cases of the successful treatment of patients with PFAPA through tonsillectomy were reported, however, the data are isolated, unstructured and need to be detailed [6].

The aim of the study wastodeterminethe frequency ofPFAPA-syndromeinchildren,who were aimed at the routine tonsillectomy, and the efficiency ofthe tonsillectomyinchildrenwith PFAPA-syndrome.

Мaterial and methods

During the 2014-2016 periods the tonsillectomy and adenotonsillectomy were performed in 216 children. The inclusion criteria in the study were the age from3 to 15 yearsoldand recurrent

tonsillopharyngitis. The exclusion criterion from the study was the peritonsillar abscess history. The rheumotests and the identification the beta- haemolytic streptococcus in the pharynx were performed to all patients before tonsillectomy to estimate the bacterial background of the disease. PFAPA-syndrome was diagnosed according to the Thomas criteria:

1. The periods of hyperthermia with certain intervals and the beginning at an early age (under 5 years old).

2. The presence of at least one of the following symptoms: aphthous stomatitis, tonsillopharyngitis with films on the tonsils, increasing of the cervical lymph nodes.

3. The exclusion of the cyclic neutropenia.

4. The absence of the symptoms between episodes of hyperthermia.

5. The normal psycho-motor development of the child.

The questionnaire was developed in case to

improve diagnostic and treatment of the chronic tonsillitis in children. It is consists of the eight questions and helps to suggest a possible etiology of the disease (Table.).

To estimate the efficacy of the tonsillectomy the amount of episodes of upper respiratory tract infection were recorded during the year after tonsillectomy as well as the amount of courses of antibiotic therapy for infections of the upper respiratory tract and the amount of days of not-attending the schools because of the throat infection.

Results and discussion

From the general amount of the 216 tonsillectomy children the 16 were excluded because of the presence of peritonsillar abscess history, 6 - because of the infectious mononucleosis, 14 of the children were refused to participate in the study.

The hemolytic streptococci were identified in 26 children (14,4%), the increasing of the titers of antistreptolysin O was diagnosed in 115 patients (63,9%), the increasing of the levels of rheumatoid factor - in 14 patients (7,8%). PFAPA-syndrome according to the criteria Thomas was diagnosed in 6 children (3,3%).

The four from the six patients with PFAPA were male (three - 3 years old and one - 6) and two of them were female of the 4 and 5 years old. The average age of patients with PFAPA was 4 years old. The average period between PFAPA-episodes was 28,0 days. The typical episodes of the tonsillopharyngitis with increased cervical lymph nodes and febrile fever with poor responsive to antipyretics and good response to steroids were revealed in each case. The average duration of hyperthermia was 4,0 days. The symptoms of the aphthous stomatitis were found in 4 children. The one PFAPA-child was examined by surgeons several times to exclude the acute abdominal pathology because of the abdominal pain and vomiting without diarrhea.

The two groups were formed according to the results of examination. The 23 children with proven bacterial genesis of chronic tonsillitis were included in the first group. These 23 children had haemolytic streptococcus in the oropharynx as well as the high titer of antistreptolysin O. The second group was formed of 6 children with symptoms that were assessed as PFAPA-syndrome.

The testing of the developed questionnaire showed that the total scores in children with proven bacterial genesis ranged from 8 to 20 points with average of 10,4 points. The total scores of the children with the PFAPA syndrome ranged from 22 to 32 points with average of 28 points.

In the first group the carrier of the hemolytic streptococci in the pharynx was revealed in 3 patients (9,1%) and 4 or more episodes of upper respiratory tract infection - in 4 children (17,4%) a year after surgery. Only in one case the episode of throat infection was accompanied with fever above 38° C. The average amount of courses of antibiotic treatment per year because of respiratory diseases has decreased from 6,4 to 1,8. The complete disappearance of the fever episodes in a year after surgery was established in five cases of PFAPA-syndrome. The one child had the febrile episodes but the interval between episodes increased from 26 to 90 days and it became easily to correct high temperature. The number of episodes of upper respiratory tract infections in this group decreased from 9,5 to 0,7; the number of courses of antibiotic treatment - from 10,3 to 1,4; the number of days not- attending the schools because of throat infection - from 68,4 to 14,8.

Conclusions

The haemolytic streptococcus as the etiologic factor in the development of chronic tonsillitis was found in 64% of children with routine tonsillectomy because of frequent episodes of tonsillopharyngitis.

The frequency of the PFAPA-syndrome among tonsillectomy children was 3,3%.

The constructedquestionnaireimprovesthe differential diagnosis process of the episodes of tonsillopharyngitis in children. Thus, the total number of points less than 10 may indicate the bacterial origin of the chronic tonsillitis, but the total number of points above 24 points indicates the high probability of PFAPA-syndrome.

Tonsillectomy is an effectivetreatmentfor children with PFAPA syndrome with disappearance of debilitating episodes of fever and tonsillopharyngitis.

Prospects for further researches

The future investigation of the aspects of the development of PFAPA-syndrome will improve the effectiveness of treatment.

References

1. Lantto U, Koivunen P, Tapiainen T, Renko M. Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy. J Pediatr. [Internet]. 2016 Dec;179:172-177. Available from: https://www.ncbi.nlm.nih.gov/pubmed/27692464 / doi: 10.1016/j.jpeds.2016.08.097.

2. El Hennawi DE, Geneid A, Zaher S, Ahmed MR. Management of recurrent tonsillitis in children. Am J Otolaryngol [Internet]. 2017 Mar 3. Available from: https://www.ncbi.nlm.nih.gov/pubmed/28385329/ doi: 10.1016/j.amjoto.2017.03.001. Gimpel C, Heinrich A, Fehrenbach H, Pfeiffer J, Pisarski P, Pohl M. Increased incidence of adenoidectomy and tonsillectomy in children with renal transplantation. Pediatr Nephrol. [Internet]. 2014 Aug;29(8):1441-50. Available from: https://www.ncbi.nlm.nih.gov/pubmed/24584665 / doi:

3. Semianchuk V.B. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (pfapa) syndrome

4. in children. Wiad Lek. [Internet]. 2017;70(1): 144-147. Available from:https://www.ncbi.nlm.nih.gov/ pubmed/28343210.

5. Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J. [Internet]. 2016 Jun 27;14(1):38. Available from: https://www.ncbi.nlm.nih.gov/ pubmed/27349388 / doi: 10.1186/s12969-016-0101-9.

6. Vigo G, Martini G, Zoppi S, Vittadello F, Zulian F. Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study. Clin Exp Rheumatol. [Internet]. 2014 Jul-Aug;32(4 Suppl 84):S156-9. Available from:

7. Thus, tonsillectomy was as effective treatment for children with chronic tonsillitis bacterial origin and children with PFAPA-syndrome.

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