Retrospective Cohort Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Feb 8, 2018; 7(1): 49-55
Published online Feb 8, 2018. doi: 10.5409/wjcp.v7.i1.49
Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center
Silvia De Pauli, Sara Lega, Serena Pastore, Domenico Leonardo Grasso, Anna Monica Rosaria Bianco, Giovanni Maria Severini, Alberto Tommasini, Andrea Taddio
Silvia De Pauli, Sara Lega, Andrea Taddio, Department of Medicine, Surgery and Health, University of Trieste, Trieste 34142, Italy
Sara Lega, Serena Pastore, Domenico Leonardo Grasso, Anna Monica Rosaria Bianco, Giovanni Maria Severini, Alberto Tommasini, Andrea Taddio, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste 34137, Italy
Author contributions: De Pauli S wrote the manuscript and cared for patients; Lega S discussed and revised critically the manuscript; Pastore S discussed and corrected the draft; Grasso DL performed ENT evaluation and revised the manuscript; Bianco AM performed genetic analyses and revised the manuscript; Severini GM reviewed the genetic content and discussed results; Tommasini A cared for patients and discussed the work; Taddio A cared for patients and critically discussed the manuscript; De Pauli S and Lega S contributed equally to the work.
Supported by the Institute for Maternal and Child Health IRCCS Burlo Garofolo, No. RC36/11.
Institutional review board statement: The study was approved by the IRB at the IRCCS Burlo Garofolo.
Informed consent statement: The written informed consent was obtained by all the subjects recruited in the study or by their parents/guardians.
Conflict-of-interest statement: The authors declare no conflict of interest.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Alberto Tommasini, MD, PhD, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Via dell’Istria 65/1, Trieste 34137, Italy.
Telephone: +39-40-3785422 Fax: +39-40-3785452
Received: September 23, 2017
Peer-review started: September 24, 2017
First decision: November 27, 2017
Revised: December 3, 2017
Accepted: December 14, 2017
Article in press: December 14, 2017
Published online: February 8, 2018
Research background

Undifferentiated periodic fevers (UPF) include periodic fever not meeting the diagnostic criteria for typical PFAPA of for hereditary periodic fever syndromes. Even if UPF are increasingly recognized, there is currently no recommendation to guide the management of children with this condition.

Research motivation

Clinical criteria to classify periodic fever without the help of genetic analyses have been proposed by PRINTO Eurofever, and might be particularly useful for subjects with UPF. Thus, we studied the clinical features of our patients in relation with their scores in the PRINTO-Eurofever classification.

Research objectives

Our study aims at improving knowledge on UPF and at evaluating if the application of the PRINTO-Eurofever classification can help the clinical management of these patients.

Research methods

A data base was filled in by retrospective review of clinical records, follow-up visits and phone calls. A structured questionnaire was used to classify all the subjects with the PRINTO-Eurofever score. The response to therapies and the prognosis at follow-up was compared with the clinical diagnosis obtained with the PRINTO-Eurofever score.

Research results

The clinical manifestations are on a half way of clinical spectrum between PFAPA and hereditary periodic fever. PRINTO-Eurofever score is not useful to guide treatment choices and does not predict disease course. Both steroids and colchicine are useful to control symptoms in most cases.

Research conclusions

UPF are as common as hereditary periodic fever, however knowledge on prognosis and response to therapies in these patients is lacking.

Research perspectives

Multicenter studies and experts’ agreement are needed to develop recommendations for the management of UPF.