PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches
Introduction: PFAPA syndrome is an autoinflammatory disease seen in childhood characterized by recurrent fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This study aims to examine the clinical and laboratory features of PFAPA syndrome, assess responses to treatment, and particularly...
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| Format: | Article |
| Language: | English |
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Galenos Publishing House
2024-08-01
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| Series: | Güncel Pediatri |
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| Online Access: | https://www.guncelpediatri.com/articles/pfapa-syndrome-evaluation-of-clinical-findings-immunological-alterations-and-treatment-approaches/doi/jcp.2024.36604 |
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| author | Şefika İlknur Kökçü Karadağ Esra Bekar Çepni Zeynep Gizem Gündüz Alişan Yıldıran |
| author_facet | Şefika İlknur Kökçü Karadağ Esra Bekar Çepni Zeynep Gizem Gündüz Alişan Yıldıran |
| author_sort | Şefika İlknur Kökçü Karadağ |
| collection | DOAJ |
| description | Introduction: PFAPA syndrome is an autoinflammatory disease seen in childhood characterized by recurrent fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This study aims to examine the clinical and laboratory features of PFAPA syndrome, assess responses to treatment, and particularly evaluate the disease from an immunological perspective.
Materials and Methods: Forty-nine patients presenting to the Pediatric Immunology and Allergy outpatient clinic and meeting the diagnostic criteria for PFAPA were evaluated. Demographic information, symptoms, physical examination findings, laboratory results, and responses to treatment were meticulously recorded.
Results: Our study included a total of 49 patients, comprising 30 males and 19 females, with a mean age at diagnosis of 3.5 years. Notable findings in immunological assessments included neutrophilia, leukocytosis, and in a few cases, lymphopenia, as well as changes in CD3 and CD19 subsets that highlighted the immunological aspect of the disease, indicating significant alterations in the adaptive immune system. Prednisolone treatment resulted in a response rate of 92.5%, with most patients showing a rapid improvement. Eighty-one point eight percent of patients receiving colchicine prophylaxis reported a decrease in symptoms. The symptoms in 13.5% of patients who underwent tonsillectomy either significantly decreased or completely resolved.
Conclusion: Management of PFAPA syndrome varies in terms of immunological findings and response rates to treatment. Our study elucidates the effectiveness of prednisolone treatment, the benefits of colchicine prophylaxis, and the improvement in symptoms following tonsillectomy. Furthermore, it underscores the necessity of considering immunological factors in the diagnosis and treatment processes of PFAPA syndrome. |
| format | Article |
| id | doaj-art-57f079ec419c46d2a9b80ffd76356602 |
| institution | Kabale University |
| issn | 1304-9054 1308-6308 |
| language | English |
| publishDate | 2024-08-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Güncel Pediatri |
| spelling | doaj-art-57f079ec419c46d2a9b80ffd763566022024-12-12T07:34:45ZengGalenos Publishing HouseGüncel Pediatri1304-90541308-63082024-08-0122212513210.4274/jcp.2024.36604PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment ApproachesŞefika İlknur Kökçü Karadağ0https://orcid.org/0000-0001-9234-633XEsra Bekar Çepni1https://orcid.org/0009-0005-0511-5637Zeynep Gizem Gündüz2https://orcid.org/0000-0003-3325-0869Alişan Yıldıran3https://orcid.org/0000-0002-2918-6238Ondokuz Mayıs University Faculty of Medicine Department of Child Allergy and Immunology, Samsun, TurkeyOndokuz Mayıs University Faculty of Medicine Department of Child Allergy and Immunology, Samsun, TurkeyOndokuz Mayıs University Faculty of Medicine Department of Child Allergy and Immunology, Samsun, TurkeyOndokuz Mayıs University Faculty of Medicine Department of Child Allergy and Immunology, Samsun, TurkeyIntroduction: PFAPA syndrome is an autoinflammatory disease seen in childhood characterized by recurrent fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This study aims to examine the clinical and laboratory features of PFAPA syndrome, assess responses to treatment, and particularly evaluate the disease from an immunological perspective. Materials and Methods: Forty-nine patients presenting to the Pediatric Immunology and Allergy outpatient clinic and meeting the diagnostic criteria for PFAPA were evaluated. Demographic information, symptoms, physical examination findings, laboratory results, and responses to treatment were meticulously recorded. Results: Our study included a total of 49 patients, comprising 30 males and 19 females, with a mean age at diagnosis of 3.5 years. Notable findings in immunological assessments included neutrophilia, leukocytosis, and in a few cases, lymphopenia, as well as changes in CD3 and CD19 subsets that highlighted the immunological aspect of the disease, indicating significant alterations in the adaptive immune system. Prednisolone treatment resulted in a response rate of 92.5%, with most patients showing a rapid improvement. Eighty-one point eight percent of patients receiving colchicine prophylaxis reported a decrease in symptoms. The symptoms in 13.5% of patients who underwent tonsillectomy either significantly decreased or completely resolved. Conclusion: Management of PFAPA syndrome varies in terms of immunological findings and response rates to treatment. Our study elucidates the effectiveness of prednisolone treatment, the benefits of colchicine prophylaxis, and the improvement in symptoms following tonsillectomy. Furthermore, it underscores the necessity of considering immunological factors in the diagnosis and treatment processes of PFAPA syndrome.https://www.guncelpediatri.com/articles/pfapa-syndrome-evaluation-of-clinical-findings-immunological-alterations-and-treatment-approaches/doi/jcp.2024.36604pfapa syndromeperiodic feverautoinflammatory diseasesimmunologyprednisolonecolchicinetonsillectomy |
| spellingShingle | Şefika İlknur Kökçü Karadağ Esra Bekar Çepni Zeynep Gizem Gündüz Alişan Yıldıran PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches Güncel Pediatri pfapa syndrome periodic fever autoinflammatory diseases immunology prednisolone colchicine tonsillectomy |
| title | PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches |
| title_full | PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches |
| title_fullStr | PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches |
| title_full_unstemmed | PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches |
| title_short | PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches |
| title_sort | pfapa syndrome evaluation of clinical findings immunological alterations and treatment approaches |
| topic | pfapa syndrome periodic fever autoinflammatory diseases immunology prednisolone colchicine tonsillectomy |
| url | https://www.guncelpediatri.com/articles/pfapa-syndrome-evaluation-of-clinical-findings-immunological-alterations-and-treatment-approaches/doi/jcp.2024.36604 |
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