ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 041

Ancestry, Demographic and Clinical Features of Israeli Periodic Fever Aphthous Stomatitis, Pharyngitis and Adenitis (PFAPA) Syndrome – a Multi-center Cohort

Gil Amarilyo1, Liora Harel 2, Sabreen Abu Ahmad 3, Maryam Abu Rumi 3, Riva Brik 4, Nofar Hezkelo 5, Orly Ohana 6, Yoel Levinsky 7, Gabriel Chodick 5 and Yonatan Butbul Aviel 8, 1Schneider Hospital, Tel Aviv University, Kibbutz Magal, Israel, 2Schneider Hospital, Tel Aviv University, Petah-Tiqva, Israel, 3Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel, 4Haifa, Israel, 5Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel, Tel Aviv, Israel, 6Schneider Children's Medical Cener of Israel, Petach Tikva, Israel, 7Schneider Children's Medical Cnetr of Israel, Tel Aviv University, Petach Tikva, Israel, 8Rambam Medical center, Haifa, Hefa, Israel

Meeting: 2020 Pediatric Rheumatology Symposium

Keywords: Autoinflammatory Disease

  • Tweet
  • Email
  • Print
Session Information

The 2020 Pediatric Rheumatology Symposium, originally scheduled for April 29 – May 2, was postponed due to COVID-19; therefore, abstracts were not presented as scheduled.

Date: Thursday, April 30, 2020

Title: Poster Session 1

Session Type: ACR Abstract Session

Session Time: 6:00PM-7:00PM

Background/Purpose: Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is an autoinflammatory disease of unknown etiology. Recently, we showed that there may be a simultaneous presentation of PFAPA and FMF. We have noted higher prevalence of PFAPA syndrome among children from Mediterranean ancestry in our clinic.  Therefore, we have conducted a multi-center clinical study which aimed to identify their ancestry and compare them to patients with asthma (which has similar prevalence among Caucasian ancestries).

Methods: Medical records of PFAPA patients from 2 tertiary Israeli medical centers were reviewed between March 2014 and March 2019 with exclusion of patients with concomitant FMF.  The Mediterranean group was defined as children from Arabic or Jewish Sephardic ancestry. Children who had both Mediterranean and Non- Mediterranean ancestry were defined as having multiethnic ancestry.

Results: Overall 303 PFAPA patients were included (after exclusion of 51 patients with concomitant FMF disease) and were compared to 475 asthmatic patients. Among PFAPA patients 17 (5.8%) were from Non-Mediterranean ancestry group – all were Ashkenazy Jews. 178 (58.7%) patients had pure Mediterranean ancestry and 96 (33.0%) had Multiethnic ancestry.  The distribution of the asthmatic patients by ancestry was similar among the 3 groups (about third in each group). PFAPA patients were significantly more likely to be from a Mediterranean ancestry compared to asthmatic patients (P< 0.0001). In addition, Mediterranean PFAPA Patients had significantly earlier disease onset and were diagnosed earlier (P< 0.04). In all PFAPA groups there was an average of delay of about 2 years from onset to diagnosis.

Conclusion: About 60% of our PFAPA patients were of Mediterranean ancestry and only 6% were of Ashkenazy ancestry. Future studies are needed to determine the genetic background of these findings.


Disclosure: G. Amarilyo, None; L. Harel, None; S. Abu Ahmad, None; M. Abu Rumi, None; R. Brik, None; N. Hezkelo, None; O. Ohana, None; Y. Levinsky, None; G. Chodick, None; Y. Butbul Aviel, None.

To cite this abstract in AMA style:

Amarilyo G, Harel L, Abu Ahmad S, Abu Rumi M, Brik R, Hezkelo N, Ohana O, Levinsky Y, Chodick G, Butbul Aviel Y. Ancestry, Demographic and Clinical Features of Israeli Periodic Fever Aphthous Stomatitis, Pharyngitis and Adenitis (PFAPA) Syndrome – a Multi-center Cohort [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 4). https://acrabstracts.org/abstract/ancestry-demographic-and-clinical-features-of-israeli-periodic-fever-aphthous-stomatitis-pharyngitis-and-adenitis-pfapa-syndrome-a-multi-center-cohort/. Accessed .
  • Tweet
  • Email
  • Print

« Back to 2020 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/ancestry-demographic-and-clinical-features-of-israeli-periodic-fever-aphthous-stomatitis-pharyngitis-and-adenitis-pfapa-syndrome-a-multi-center-cohort/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology