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Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review.
Clin Rheumatol. 2019 Jan; 38(1):11-17.CR

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed before 5 years, though reports regarding adulthood are increasing. The pathogenesis of the syndrome is not completely understood, but a multifactorial origin, probably based on a polygenic pattern of susceptibility, is the most probable rational pathogenetic hypothesis. Treatment of PFAPA syndrome relies on the administration of low-dose corticosteroids, which promptly abort flares but cannot prevent subsequent disease episodes over time. Tonsillectomy with or without adenoidectomy has proved to be successful in some pediatric patients, as proven by different studies. On the other hand, colchicine, cimetidine, nonsteroidal anti-inflammatory drugs, and interleukin-1 inhibitors have shown efficacy, which require further definite confirmations. This review is aimed at summarizing all the recent evidence about treatment options available for PFAPA syndrome both in pediatric and adult patients.

Authors+Show Affiliations

Institute of Pediatrics, University of Siena, Siena, Italy.Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy. Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.Institute of Pediatrics, University of Siena, Siena, Italy.Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. cantariniluca@hotmail.com. Policlinico "Le Scotte", Viale Bracci 1, 53100, Siena, Italy. cantariniluca@hotmail.com.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

30488366

Citation

Gaggiano, Carla, et al. "Treatment Options for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome in Children and Adults: a Narrative Review." Clinical Rheumatology, vol. 38, no. 1, 2019, pp. 11-17.
Gaggiano C, Rigante D, Sota J, et al. Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clin Rheumatol. 2019;38(1):11-17.
Gaggiano, C., Rigante, D., Sota, J., Grosso, S., & Cantarini, L. (2019). Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clinical Rheumatology, 38(1), 11-17. https://doi.org/10.1007/s10067-018-4361-2
Gaggiano C, et al. Treatment Options for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome in Children and Adults: a Narrative Review. Clin Rheumatol. 2019;38(1):11-17. PubMed PMID: 30488366.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. AU - Gaggiano,Carla, AU - Rigante,Donato, AU - Sota,Jurgen, AU - Grosso,Salvatore, AU - Cantarini,Luca, Y1 - 2018/11/28/ PY - 2018/09/27/received PY - 2018/11/05/accepted PY - 2018/11/30/pubmed PY - 2019/5/14/medline PY - 2018/11/30/entrez KW - Aphthous stomatitis KW - Autoinflammation KW - Cimetidine KW - Colchicine KW - Corticosteroids KW - Familial Mediterranean fever KW - Innovative biotechnologies KW - Interleukin-1 KW - MEFV KW - Nonsteroidal anti-inflammatory drugs KW - Periodic fever KW - Personalized medicine KW - Pharyngitis and cervical adenitis (PFAPA) syndrome KW - Tonsillectomy SP - 11 EP - 17 JF - Clinical rheumatology JO - Clin. Rheumatol. VL - 38 IS - 1 N2 - Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed before 5 years, though reports regarding adulthood are increasing. The pathogenesis of the syndrome is not completely understood, but a multifactorial origin, probably based on a polygenic pattern of susceptibility, is the most probable rational pathogenetic hypothesis. Treatment of PFAPA syndrome relies on the administration of low-dose corticosteroids, which promptly abort flares but cannot prevent subsequent disease episodes over time. Tonsillectomy with or without adenoidectomy has proved to be successful in some pediatric patients, as proven by different studies. On the other hand, colchicine, cimetidine, nonsteroidal anti-inflammatory drugs, and interleukin-1 inhibitors have shown efficacy, which require further definite confirmations. This review is aimed at summarizing all the recent evidence about treatment options available for PFAPA syndrome both in pediatric and adult patients. SN - 1434-9949 UR - https://www.unboundmedicine.com/medline/citation/30488366/Treatment_options_for_periodic_fever_aphthous_stomatitis_pharyngitis_and_cervical_adenitis__PFAPA__syndrome_in_children_and_adults:_a_narrative_review_ L2 - https://dx.doi.org/10.1007/s10067-018-4361-2 DB - PRIME DP - Unbound Medicine ER -