Periodic fever syndromes.
The term periodic fever syndrome has been used in a restricted sense to denote two diseases in which episodic fevers occur with a regular periodicity: cyclic neutropenia and the periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) syndrome. Other authors have used the term in a more general sense to encompass a larger group of disorders characterized by recurrent episodes of fever that do not necessarily follow a strictly periodic pattern. These include familial Mediterranean fever, the autosomal dominant familial fevers (also known as Hibernian fever), and the hyperimmunoglobulin D syndrome. This article follows the latter usage, and reviews recent advances in our understanding of the genetics and molecular pathology of this group of diseases, as well as their clinical characterization and treatment.
Scholl
P R
PR
Department of Pediatrics, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois 60614, USA. prscholl@northwestern.edu
D002648:Child; D010505:Familial Mediterranean Fever; D005334:Fever; D006801:Humans; D010507:Periodicity