Published online Feb 8, 2018. doi: 10.5409/wjcp.v7.i1.49
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
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.
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.
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.
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.
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.
UPF are as common as hereditary periodic fever, however knowledge on prognosis and response to therapies in these patients is lacking.
Multicenter studies and experts’ agreement are needed to develop recommendations for the management of UPF.