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Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome?
Paediatr Drugs. 2020 Jun; 22(3):243-250.PD

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever flares accompanied by symptoms and signs forming the disease acronym and alternating with asymptomatic periods. Despite the disease having a generally favorable outcome, with spontaneous remission after a few years, it does have a major impact on the quality of life of the child and his or her relatives. Beside symptomatic medications during fever flares, the most used treatment consists of a single dose of corticosteroids at flare onset to interrupt the attack; fever resolves usually within a few hours, but often with a shorter interval between the attacks. For these patients, colchicine has been shown to decrease the frequency of the flares. Other medications were also reported in case series of patients with PFAPA syndrome. These include the interleukin-1 blocker anakinra to treat flares, cimetidine (which showed no convincing effect), and other drugs with anecdotal use. The pediatrician faces a difficult question: should they wait for spontaneous disease resolution and only treat the flares medically, or should they propose tonsillectomy that may induce remission? Due to the lack of strong evidence, the answer will vary on an individual basis, depending on the impact on the patient's quality of life. The choice of the best therapeutic strategy will be based on the response to the flare-based therapy and on disease severity (quality of life, school performances); long-term data on the remission rate under the different therapeutic strategies are currently missing. More randomized controlled studies are needed to help the treating physician to choose the best therapy, as well as real-life long-term data to evaluate the long-term outcome of children with PFAPA syndrome.

Authors+Show Affiliations

Unité Romande d'immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne, and HUG, Geneva, Switzerland. michael.hofer@chuv.ch.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32342289

Citation

Hofer, Michaël. "Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome?" Paediatric Drugs, vol. 22, no. 3, 2020, pp. 243-250.
Hofer M. Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome? Paediatr Drugs. 2020;22(3):243-250.
Hofer, M. (2020). Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome? Paediatric Drugs, 22(3), 243-250. https://doi.org/10.1007/s40272-020-00393-4
Hofer M. Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome. Paediatr Drugs. 2020;22(3):243-250. PubMed PMID: 32342289.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome? A1 - Hofer,Michaël, PY - 2020/4/29/pubmed PY - 2020/7/28/medline PY - 2020/4/29/entrez SP - 243 EP - 250 JF - Paediatric drugs JO - Paediatr Drugs VL - 22 IS - 3 N2 - Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever flares accompanied by symptoms and signs forming the disease acronym and alternating with asymptomatic periods. Despite the disease having a generally favorable outcome, with spontaneous remission after a few years, it does have a major impact on the quality of life of the child and his or her relatives. Beside symptomatic medications during fever flares, the most used treatment consists of a single dose of corticosteroids at flare onset to interrupt the attack; fever resolves usually within a few hours, but often with a shorter interval between the attacks. For these patients, colchicine has been shown to decrease the frequency of the flares. Other medications were also reported in case series of patients with PFAPA syndrome. These include the interleukin-1 blocker anakinra to treat flares, cimetidine (which showed no convincing effect), and other drugs with anecdotal use. The pediatrician faces a difficult question: should they wait for spontaneous disease resolution and only treat the flares medically, or should they propose tonsillectomy that may induce remission? Due to the lack of strong evidence, the answer will vary on an individual basis, depending on the impact on the patient's quality of life. The choice of the best therapeutic strategy will be based on the response to the flare-based therapy and on disease severity (quality of life, school performances); long-term data on the remission rate under the different therapeutic strategies are currently missing. More randomized controlled studies are needed to help the treating physician to choose the best therapy, as well as real-life long-term data to evaluate the long-term outcome of children with PFAPA syndrome. SN - 1179-2019 UR - https://www.unboundmedicine.com/medline/citation/32342289/Why_and_How_Should_We_Treat_Periodic_Fever,_Aphthous_Stomatitis,_Pharyngitis,_and_Cervical_Adenitis_(PFAPA)_Syndrome L2 - https://dx.doi.org/10.1007/s40272-020-00393-4 DB - PRIME DP - Unbound Medicine ER -