title: Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part II.
abstract: Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for approaching patients with periodic fever and reviewed two common disorders with periodic fever in Iranian patients including familial Mediterranean fever (FMF) and periodic fever syndromes except for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA). In this part, we review other autoinflammatory disorders including hyper IgD, tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin associated periodic syndromes, autoinflammatory bone disorders and some other rare autoinflammatory disorders such as Sweet's and Blau syndromes. In cryopyrin associated periodic syndromes group, we discussed chronic infantile neurologic cutaneous and articular (CINCA) syndrome, Muckle-Wells syndrome and familial cold autoinflammatory syndrome. Autoinflammatory bone disorders are categorized to monogenic disorders such as pyogenic arthritis, pyoderma ;gangraenosum and acne (PAPA) syndrome, the deficiency of interleukine-1 receptor antagonist (DIRA) and Majeed syndrome and polygenic background or sporadic group such as chronic recurrent multifocal osteomyelitis (CRMO) or synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome are classified in sporadic group. Other autoinflammatory syndromes are rare causes of periodic fever in Iranian system registry.
authors.affiliation: Department of Infectious Diseases, Imam Khomeini Hospital.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Ahmadinejad
authors.affiliation: Department of Infectious Diseases, Imam Khomeini Hospital.
authors.forename: Sedigeh
authors.initials: S
authors.lastname: Mansouri
authors.affiliation: Pediatric Rheumatology Research Group, Rheumatology Research Center ; Department of Pediatrics ; Children's Medical Center, Pediatrics Center of Excellence.
authors.forename: Vahid
authors.initials: V
authors.lastname: Ziaee
authors.affiliation: Department of Pediatrics.
authors.forename: Yahya
authors.initials: Y
authors.lastname: Aghighi
authors.affiliation: Department of Pediatrics ; Growth & Development Research Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad-Hassan
authors.initials: MH
authors.lastname: Moradinejad
authors.affiliation: Children's Medical Center, Pediatrics Center of Excellence.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Fereshteh-Mehregan
keywords: Periodic Fever; Hyper IgD; TRAPS; Autoinflammatory Bone Disorder; Cryopyrin Associated Periodic Syndromes; Sweet’s Syndrome; Blau Syndrome