Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part II

authors:

avatar Zahra Ahmadinejad 1 , avatar Sedigeh Mansouri 2 , avatar Vahid Ziaee 3 , * , avatar Yahya Aghighi 4 , avatar Mohammad-Hassan Moradinejad 5 , avatar Fatemeh- Fereshteh Mehregan 6

Iranian Journal of Pediatrics: Vol.24, issue 3; 229-240
published online: May 05, 2014
article type: Review Article
received: September 20, 2013
accepted: January 03, 2014

how to cite: Ahmadinejad Z , Mansouri S , Ziaee V, Aghighi Y , Moradinejad M , et al. Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part II. Iran J Pediatr. 2014;24(3): 229-240. 

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.

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