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  • Rise in children presenting with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome during the COVID-19 pandemic

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Rise in children presenting with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome during the COVID-19 pandemic
  1. Khuen Foong Ng1,
  2. Joseph Morgan2,
  3. Thomas Cutts2,
  4. Isabel Duncan2,
  5. Marion Roderick1,3,
  6. Athimalaipet Ramanan4,5,
  7. Anu Goenka1,6
  1. 1 Paediatric Infectious Diseases and Immunology, Bristol Royal Hospital for Children, Bristol, UK
  2. 2 Paediatrics, Bristol Royal Hospital for Children, Bristol, UK
  3. 3 Bristol Medical School, University of Bristol, Bristol, UK
  4. 4 Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK
  5. 5 Translational Health Science, University of Bristol, Bristol, UK
  6. 6 Cellular and Molecular Medicine, University of Bristol, Bristol, UK
  1. Correspondence to Dr Anu Goenka, Bristol Medical School, Bristol BS8 1TD, UK; anu.goenka{at}bristol.ac.uk

https://doi.org/10.1136/archdischild-2021-322792

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    Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is characterised by episodes of fever lasting a few days that classically exhibit clockwork periodicity. Since the initial description of PFAPA syndrome by Gary Marshall in 1987, it has been recognised that stomatitis, pharyngitis and adenitis are variably present.1 Its phenotype is consistent with an autoinflammatory condition of unknown genetic aetiology possibly involving an infectious/environmental trigger, given that a family history is present in approximately 27% of cases.2 The natural history is onset before 6 years old, followed by spontaneous resolution by 15 years. Treatment with colchicine can reduce the frequency of episodes and tonsillectomy is usually curative.3

    The diagnosis of PFAPA syndrome is clinical but can be challenging because it predominantly affects young children who typically experience frequent febrile viral infections. We hypothesised that reduced transmission of viruses due to COVID-19 public health control measures …

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    Footnotes

    • Twitter @anu_goenka

    • Contributors AG, MR and AR conceptualised, reviewed and revised the manuscript. KFN and AG designed the data collection instruments, coordinated and supervised data collection, carried out the initial analyses, drafted, reviewed and revised the manuscript. JM, TC and ID collected data, reviewed and revised the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; internally peer reviewed.