Periodic Fever: A Review on Clinical and Management Aspects and Epidemiologic Characteristics in Iran

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. The first manifestation of these disorders are present in childhood and adolescence, but an infrequently it may be presented in young and middle ages. Genetics base has been known for all type of periodic fever syndromes except periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). Common periodic fever disorders are Familial Mediterranean fever (FMF) and PFAPA. In each patient with periodic fever, acquired infection with chronic and periodic nature should be ruled out. It is depend on epidemiology of infectious diseases. Some of them are common in Iran such as Familial Mediterranean fever and PFAPA. In Iran and other Middle East countries, brucellosis, Malaria and infectious mononucleosis should be considered in differential diagnosis of periodic fever disorders especially with fever and arthritis manifestation. In children, urinary tract infection may be presented as periodic disorder, urine analysis and culture is necessary in each child with periodic symptoms. Some malignancies such as leukemia and tumoral lesion should be excluded in each patient with periodic syndromes and weight loss in each age. After exclude infection, malignancy and cyclic neutropenia, FMF and PFAPA are the most common periodic fever disorders. Similar other countries, Hyper IgD, Chronic Infantile Neurologic Cutaneous and Articular, TRAPS and other authoinflamatory syndromes are rare causes of periodic fever disorders in our system registry of periodic fever in Iran. In the part 1 of this paper we reviewed FMF and PFAPA regarding to the prevalence of them in Iran. In the second part, some uncommon auto-inflammatory disorders such as TRAPS, Hyper IgD sydroms and cryopyrin associated periodic syndromes will be reviewed.