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Correspondence response
Response to: ‘Do we need the PFAPA syndrome in adults with non-monogenic periodic fevers?’ by Fayand et al
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  1. Marco Gattorno1,
  2. Michael Hofer2,
  3. Federica Vanoni3,
  4. Silvia Federici4,
  5. Luca Cantarini5,
  6. Nicolino Ruperto6
  7. for the Paediatric Rheumatology International Trials Organisation (PRINTO) and the Eurofever Registry
  1. 1 Istituto Giannina Gaslini, Genova, Italy
  2. 2 Department of Paediatrics, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland
  3. 3 Ospedale Regionale di Bellinzona e Valli, Bellinzona, Switzerland
  4. 4 Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
  5. 5 Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, University of Siena, Siena, Italy
  6. 6 Pediatria II—PRINTO, IRCCS G Gaslini, Genova, Italy
  1. Correspondence to Marco Gattorno, Istituto Giannina Gaslini, Genova 16147, Italy; marcogattorno{at}gaslini.org

https://doi.org/10.1136/annrheumdis-2019-216862

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    We really thank Fayand and coworkers for their interest to the new Eurofever/PRINTO classification criteria for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA)1 and for their interesting exercise to apply the three most recent criteria to their population of adult patients with recurrent fever fulfilling the revised Marshall’s criteria for PFAPA.2

    Indeed, in the last few years, three new criteria for PFAPA have been proposed.3–5 Of note, the methodology used for the development of the three set of criteria was rather different, as is for the process of their validation in an independent population.

    Cantarini’s criteria were exclusively developed for adult-onset PFAPA patients. Seventy-four adult patients fulfilling the modified Marshall’s criteria were compared with 62 patients with fever of unknown origin (FUO). A multivariate analysis identified the set of variables with the highest accuracy in distinguishing PFAPA from FUO patients.4 The criteria have not been validated in an independent population, so far.

    Vanoni’s criteria were created using the standard consensus procedures (Delphi survey and Nominal Group Technique in a Consensus Conference) among 22 paediatric experts in autoinflammation. The new proposed criteria were tested in 80 paediatric PFAPA patients followed in two centres for autoinflammatory diseases (Genoa and Lausanne). For this validation process, the diagnosis of PFAPA was not done on the basis of modified Marshall’s criteria, but on the clinical judgement of the two centres. Notably, only …

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