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
Core tip: Children with non-infectious recurrent fever more often fall into two diagnostic categories. The first and most common is periodic fever, aphthae, pharingitis, adenitis (PFAPA), the second, far more rare, are hereditary periodic fevers. Very recently a third category has been increasingly recognized, and is that of undifferentiated periodic fevers or undifferentiated periodic fever (UPF). UPF include patients who do not meet the diagnostic criteria for PFAFA or for a monogenic disease. The clinical presentation and management of patients with UPF are poorly defined. In this study, the authors describe a cohort of patients with UPF showing that: (1) The clinical manifestations are on a half way of clinical spectrum between PFAPA and hereditary periodic fever; (2) PRINTO-Eurofever score is not useful to guide treatment choices and does not predict disease course; and (3) Both steroids and colchicine are useful to control symptoms in most cases. The authors conclude that further studies are needed to better define UPF and guide their management in clinical practice.