title: Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR).
abstract: OBJECTIVE
  The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longtime sequel was reported in this disease. Early diagnosis can lead physicians to treatment of this disorder with a short course steroid application and provide satisfaction of the patient's family.
  
  
  METHODS
  This study is a prospective review of patients diagnosed with PFAPA syndrome who were registered in Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR) through periodic fever clinic in the Children's Medical Center, Pediatric Center of Excellence in Tehran, Iran from January 2013 to March 2014.
  
  
  RESULTS
  One hundred thirty patients were registered in our databases. Twenty-one (16.1%) patients including 15 males and 6 females had PFAPA. Normal growth was seen in all patients. The median age at onset was 18 months. The mean duration of fever was 4 days and the mean duration of intervals between fever episodes 21 days. Along with fever, all patients had characteristic symptoms. All patients were asymptomatic between fever episodes. Steroid was used in all patients and causing immediate reduction by 84.61%. Two patients received both steroid and colchicine because of their clinical feature and positive laboratory tests for PFAPA and familial Mediterranean fever. No patient received biological therapy or a tonsillectomy.
  
  
  CONCLUSIONS
  The long diagnostic delay of PFAPA gives cause to concern indicating a need for greater awareness of the disease so that the diagnosis may be made timely.
authors: Children's Medical Center, Pediatrics Center of Excellence ; Shahid Beheshti University of Medical Sciences.
authors: Fatemeh Fereshteh
authors: FF
authors: Mehregan
authors: Pediatric Rheumatology Research Group, Rheumatology Research Center ; Department of Pediatrics.
authors: Vahid
authors: V
authors: Ziaee
authors: Department of Infectious Diseases, Tehran University of Medical Sciences, Tehran, Iran.
authors: Zahra
authors: Z
authors: Ahmadinejad
authors: Children's Medical Center, Pediatrics Center of Excellence ; Department of Pediatrics.
authors: Fatemeh
authors: F
authors: Tahghighi
authors: Children's Medical Center, Pediatrics Center of Excellence ; Department of Pediatrics.
authors: Farah
authors: F
authors: Sabouni
authors: Children's Medical Center, Pediatrics Center of Excellence ; Department of Pediatrics.
authors: Mohamad-Hassan
authors: MH
authors: Moradinejad
keywords: Autoinflammatory Disorders; Children; PFAPA syndrome; Periodic fever; Recurrent fever