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THU0509 First 18 Month Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)
  1. F. Mehregan1,
  2. V. Ziaee2,
  3. M.H. Moradinejad2,
  4. Y. Aghighi2,
  5. S.R. Raeeskarimi2
  1. 1Pediatric Rheumatology, Shahid Beheshti University
  2. 2Pediatric Rheumatology, Tehran University, Tehran, Iran, Islamic Republic Of


Objectives The term Auto-inflammatory diseases (AIDs) has been used to describe a group of illness characterized by attacks of seemingly unprovoked inflammation without a significant level of either autoantibodies or antigen-specific T cells more characteristic of autoimmune disease. Published data from Iran regarding autoinflammatory disease in children are scarce. In a multicenter effort, we set out to register genetic, epidemiological and clinical features as well as prognostic factors of these diseases by prospective longitudinal and long-term documentation, in order to define current and novel AIDs and to better understand treatment responses and outcome.

Methods This study is a prospective cross sectional study of patients diagnosed with Auto-inflammatory diseases (AIDs) who was registered in the autoinflammatory computer database registration through periodic fever clinic, which was established for this reason in the medical center of children in 23.09.2012 in Tehran- Iran and data were collected with standardized forms. Inclusion criteria are disease-associated mutations for hereditary periodic fever syndromes [FMF, hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS), TNF receptor 1-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndrome (CAPS)], or, alternatively, clinically confirmed AID, and periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome with the unknown genetic background.

Results During the first 18 months, 139 patients (93 males, 46 females) from 27 provinces and 11 ethnicities; age (newborn to 18 years) have been recorded and classified as FMF (n=102), PFAPA (n=29), HIDS (n=1), clinically confirmed AID (n=6) including TRAPS (n=1) and CAPS (n=2); CRMO (n=2), DIRA (n=1), and sweet syndrome (n=1). The most common AID in our study were FMF and then PFAPA, CRMO, CAPS. The most common ethnicity was Turkish and Kurdish people. In total 139 patients, genetic analysis was performed in 103 patients, 88.37% have genetically confirmed disease.

Conclusions Recruitment and follow-up of patients with AID will enable us to comprehensively address the correlation between clinical and epidemiological data, genetics.


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  2. A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 2012 Jan-Feb;32(1):43-8.

Disclosure of Interest None declared

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