Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India
BackgroundReports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher incidence of infectious illnesses and significant resource constraints. Herein, we presen...
| Main Authors: | , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2022-09-01
|
| Series: | Frontiers in Immunology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.958473/full |
| _version_ | 1811267040914178048 |
|---|---|
| author | Aaqib Zaffar Banday Vibhu Joshi Kanika Arora Rohit Sadanand Suprit Basu Rakesh Kumar Pilania Ankur Kumar Jindal Pandiarajan Vignesh Anju Gupta Saniya Sharma Manpreet Dhaliwal Amit Rawat Surjit Singh Deepti Suri |
| author_facet | Aaqib Zaffar Banday Vibhu Joshi Kanika Arora Rohit Sadanand Suprit Basu Rakesh Kumar Pilania Ankur Kumar Jindal Pandiarajan Vignesh Anju Gupta Saniya Sharma Manpreet Dhaliwal Amit Rawat Surjit Singh Deepti Suri |
| author_sort | Aaqib Zaffar Banday |
| collection | DOAJ |
| description | BackgroundReports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher incidence of infectious illnesses and significant resource constraints. Herein, we present our experience from North India regarding the diagnosis and management of PFAPA syndrome.MethodsWe reviewed cases of non-monogenic periodic fever syndrome diagnosed at our center from January 2011 to December 2021. A total of 17 children who fulfilled the Marshall criteria for PFAPA syndrome were included. Data regarding basic clinical features, treatment/outcome, and performance of the recently proposed Eurofever/PRINTO and Takeuchi criteria were analyzed.ResultsBesides recurrent fever, the triad of oral aphthae, pharyngitis, and adenitis was noted in only 18% of patients. Episodes of exudative pharyngitis/tonsillitis were documented in 24%. These figures were lower than the values reported from developed countries. The Takeuchi and Eurofever/PRINTO criteria were fulfilled in 76% and 71% cases, respectively. In addition to antipyretics and supportive care, intermittent steroid therapy was the main treatment modality used. Additional treatment with colchicine (n = 3) and thalidomide (n = 1) was used successfully in a few patients. Before the diagnosis of PFAPA, all patients had received multiple courses of antimicrobials (without microbiological confirmation). These included multiple courses of antibacterials for fever, pharyngotonsillitis, and/or cervical adenitis in all patients and antivirals for fever and aphthous stomatitis in a patient. Empiric antitubercular therapy had also been administered in two patients.ConclusionsA significant proportion of patients with PFAPA seem to remain undiagnosed in the Indian subcontinent. Increased awareness and improvement in basic healthcare facilities are crucial in enhancing the recognition of PFAPA, which would eliminate the unprecedented scale of undesirable antimicrobial use in such children. |
| first_indexed | 2024-04-12T20:54:12Z |
| format | Article |
| id | doaj.art-f54ad280e6a849ce9f54f1937062cd68 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-12T20:54:12Z |
| publishDate | 2022-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-f54ad280e6a849ce9f54f1937062cd682022-12-22T03:17:02ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-09-011310.3389/fimmu.2022.958473958473Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North IndiaAaqib Zaffar BandayVibhu JoshiKanika AroraRohit SadanandSuprit BasuRakesh Kumar PilaniaAnkur Kumar JindalPandiarajan VigneshAnju GuptaSaniya SharmaManpreet DhaliwalAmit RawatSurjit SinghDeepti SuriBackgroundReports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher incidence of infectious illnesses and significant resource constraints. Herein, we present our experience from North India regarding the diagnosis and management of PFAPA syndrome.MethodsWe reviewed cases of non-monogenic periodic fever syndrome diagnosed at our center from January 2011 to December 2021. A total of 17 children who fulfilled the Marshall criteria for PFAPA syndrome were included. Data regarding basic clinical features, treatment/outcome, and performance of the recently proposed Eurofever/PRINTO and Takeuchi criteria were analyzed.ResultsBesides recurrent fever, the triad of oral aphthae, pharyngitis, and adenitis was noted in only 18% of patients. Episodes of exudative pharyngitis/tonsillitis were documented in 24%. These figures were lower than the values reported from developed countries. The Takeuchi and Eurofever/PRINTO criteria were fulfilled in 76% and 71% cases, respectively. In addition to antipyretics and supportive care, intermittent steroid therapy was the main treatment modality used. Additional treatment with colchicine (n = 3) and thalidomide (n = 1) was used successfully in a few patients. Before the diagnosis of PFAPA, all patients had received multiple courses of antimicrobials (without microbiological confirmation). These included multiple courses of antibacterials for fever, pharyngotonsillitis, and/or cervical adenitis in all patients and antivirals for fever and aphthous stomatitis in a patient. Empiric antitubercular therapy had also been administered in two patients.ConclusionsA significant proportion of patients with PFAPA seem to remain undiagnosed in the Indian subcontinent. Increased awareness and improvement in basic healthcare facilities are crucial in enhancing the recognition of PFAPA, which would eliminate the unprecedented scale of undesirable antimicrobial use in such children.https://www.frontiersin.org/articles/10.3389/fimmu.2022.958473/fulladenitisaphthous stomatitisperiodic feverPFAPApharyngitisthalidomide |
| spellingShingle | Aaqib Zaffar Banday Vibhu Joshi Kanika Arora Rohit Sadanand Suprit Basu Rakesh Kumar Pilania Ankur Kumar Jindal Pandiarajan Vignesh Anju Gupta Saniya Sharma Manpreet Dhaliwal Amit Rawat Surjit Singh Deepti Suri Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India Frontiers in Immunology adenitis aphthous stomatitis periodic fever PFAPA pharyngitis thalidomide |
| title | Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India |
| title_full | Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India |
| title_fullStr | Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India |
| title_full_unstemmed | Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India |
| title_short | Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India |
| title_sort | challenges in the diagnosis of periodic fever aphthous stomatitis pharyngitis and adenitis syndrome in developing countries a decade of experience from north india |
| topic | adenitis aphthous stomatitis periodic fever PFAPA pharyngitis thalidomide |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.958473/full |
| work_keys_str_mv | AT aaqibzaffarbanday challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT vibhujoshi challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT kanikaarora challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT rohitsadanand challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT supritbasu challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT rakeshkumarpilania challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT ankurkumarjindal challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT pandiarajanvignesh challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT anjugupta challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT saniyasharma challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT manpreetdhaliwal challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT amitrawat challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT surjitsingh challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia AT deeptisuri challengesinthediagnosisofperiodicfeveraphthousstomatitispharyngitisandadenitissyndromeindevelopingcountriesadecadeofexperiencefromnorthindia |