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 infrequently it may be presented in young and middle ages. Genetic base has been known for all types 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 depends on epidemiology of
infectious diseases. Some of them such as
Familial Mediterranean fever and PFAPA are common in Iran. 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 lesions should be excluded in patients with periodic syndrome and
weight loss in any age. After excluding
infection,
malignancy and
cyclic neutropenia, FMF and PFAPA are the most common periodic
fever disorders. Similar to other countries, Hyper
IgD, Chronic Infantile Neurologic Cutaneous and Articular, TRAPS and other auto-inflammatory syndromes are rare causes of periodic
fever in Iranian system registry. In part 1 of this paper we reviewed the prevalence of FMF and PFAPA in Iran. In part 2, some uncommon auto-inflammatory disorders such as TRAPS, Hyper
IgD sydrome and
cryopyrin associated periodic syndromes will be reviewed.