* Corresponding Author; Address: Division of Pediatric Rheumatology, Children’s Medical Center, No 62, Dr Gharib St., Keshavarz Blvd, Tehran, Iran E-mail: ziaee@tums.ac.ir © ʹͲͳ͵ by Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, All rights reserved. Iran J Pediatr; Vol 24 (No 1), Feb 2014 Published by: Tehran University of Medical Sciences (http://ijp.tums.ac.ir) Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part I Zahra Ahmadinejad 1 , MD; Sedigeh Mansori 1 , MD; Vahid Ziaee* 2,3,4 , MD; Neda Alijani 1 , MD; Yahya Aghighi 3 , MD; Nima Parvaneh 5,3 , MD; Mohammad-Hassan Mordinejad 6,3 , MD 1 Department of Infectious Diseases, Imam Khomeini Hospital, 2 Pediatric Rheumatology Research Group, Rheumatology Research Center, 3 Department of Pediatrics, Tehran University of Medical Sciences, 4 Children’s Medical Center, Pediatrics Center of Excellence, 5 Pediatric Infectious Diseases Research Center, 6 Growth & Development Research Center, Tehran University of Medical Sciences, Tehran, Iran Received: Jan 10, 2013; Accepted: Sep 13, 2013; First Online Available: Nov 16, 2013 Abstract 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. Iranian Journal of Pediatrics, Volume 24 (Number 1), February 2014, Pages: 1-13 Key Words: Periodic Fever; Familial Mediterranean Fever; PFAPA; Hyper IgD; TRAPS Introduction Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. These disorders are self-limited and improve spontaneously without specific therapy. Episodes of fever are usually associated with presence of inflammation in different parts of the body such as peritoneum, pleural spaces, testis, etc. with elevated serum acute phase reactants level. These diseases should be differentiated from infections, malignancies and other autoimmune disorders [1] . Existence of family history of periodic fever and Clinical Approach Iran J Pediatr Feb 2014; Vol 24 (No 1), Pp: 1-13