Periodic fever syndrome: Case series

Abstract

Periodic fever syndrome (PFS) is clinically characterized by repeated and variable episodes of systemic inflammation in which there is no infectious, neoplastic cause or evidence of autoantigens or autoantibodies. Symptoms alternate with periods of total remission. The cardinal clinical manifestation is the presence of fever, which is accompanied by systemic symptoms (arthritis, serositis, exanthema and ocular involvement) and that they are similar between one crisis and another, lasting days to weeks1.They present as general characteristics, childhood onset and self-limited duration of symptoms2.PFS is in group VII of the classification ofprimary immunodeficiencies3,in which it is important to differentiate the diseases linked to monogenic mutations, with complex prognosis, from the benign forms called "periodic fever, aphthous, pharyngitis and adenopathy" (PFAPA)4-5. The criteria established by Eurofever Classification Criteria (ECC)6are used to classify patients with PFS and for the benignforms, the Thomas criteria5