IEI Newsletter Report, December 2018

December 1, 2018Inborn Errors of Immunity Committee (IEI)

The IUIS Expert committee on Inborn Errors of Immunity has been very active recently. Several of our committee members have attended Summer Schools a teaching Faculty. These have included the ESID (European Society for Immunodeficiencies) Summer School (held in Pisa, Italy, May 2018 – Kate Sullivan), the Diagnostic School in Primary Immunodeficiencies School (held in Cincinnati, Ohio, US, July 2018 – Troy Torgerson), the Latin American Society for Immunodeficiencies (held in Medellin, Columbia, August 2018 – Jose Luis Franco, Stuart Tangye), and a Clinical Immunology Workshop held as part of the Australasian Society for Immunology annual conference (Perth, WA, Australia, Dec 2019 – Stuart Tangye). Our committee was also very well represented at the recent 18th Biennial meeting of ESID, held in Lisbon, Portugal, October 2018. Here, Jean-Laurent Casanova giving the opening Keynote address, Capucine Picard and Kate Sullivan gave presentations in the Educational Sessions, Capucine Picard and Steve Holland gave Plenary presentations, and Steve Holland (3 presentations), Talal Chatila, Capucine Picard, Kate Sullivan (2 presentations), and Stuart Tangye (2 presentations) all presented in parallel symposia sessions.

We also took advantage of many of the committee members attending the ESID meeting to hold an IUIS Committee meeting. This was well attended, with 15 committee members available. Several members have indicated that they will be stepping down from the committee over the next few months. So I would like to take this opportunity to thank Mimi Tang, Hans Ochs, Talal Chatila and Amos Etzioni for their invaluable contributions to the IUIS Expert Committee over the past several years. One of the key agenda items of this recent meeting was identifying and recruiting appropriate new committee members to replace these outgoing individuals, and to also make sure we strive to have a committee with sufficient geographical, expert and gender representation and composition. New members of the committee will be announced in future IUIS newsletter reports.The Expert committee on Inborn Errors of Immunity is looking to leverage its relationships with IUIS to advance our activities. There are two major opportunities through which we are working to achieve this. First, interact with other IUIS committees, the most obvious ones would be the Clinical Immunology Committee, Publications Committee, Education Committee, and Gender Equality & Career Development Committee. As chair, I am in the process of establishing dialogues with the chairs of these other committees. Second (which is an extension of one of the firsts) is to contribute to publications by Frontiers in Immunology, as it is recognised as the official journal of IUIS. Fortunately, we are well positioned to achieve this, with an opportunity to contribute to a Research Topic on “Applications of Flow Cytometry to Primary Immunodeficiencies”.

The next major objective of the IUIS Expert committee on Inborn Errors of Immunity is the organization of the 2019 face-to-face meeting. This meeting will be held at Rockefeller University in New York just before the annual Henry Kunkel Society meeting in March 2019. The goal of this meeting will be for us to review all of the recently-published genetic defects underlying immune dysregulatory conditions in order to collate these into the next Phenotypic and Genetic classifications of primary immunodeficiencies/inborn errors of immunity, which will be published in late 2019/early 2020. There are currently 354 inborn errors of immunity, due to loss or gain of function mutations in ~330 genes. With the explosion in the rate of detection of gene defects underlying monogenic inborn errors of immune dysregulation by next generation sequencing technologies, the number of genetic etiologies will easily exceed 400. So the committee will be very busy discussing these findings.Additional upcoming activities for the IUIS Expert Committee and its members on Inborn Errors of Immunity includes:

  • Working with the Clinical immunology committee to support educational activities 
  • educational initiatives, including participation in the 2019 Clinical Immunology Society (CIS) annual meeting and pre-conference education day;
  • attendance at the IPOPI (International Patient Organisation for Primary Immunodeficiency)/IPIC (International Primary Immunodeficiency Congress) meeting to be held in Madrid in November 2019;
  • continuing liaising with IUIS to ensure participation and representation of our committee members, as well as an appropriate symposium session on Inborn errors of immunity, at the IUIS meeting to be held in Beijing in 2019.

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