IEI Newsletter Contribution, July 2019

July 1, 2019Inborn Errors of Immunity Committee (IEI)

It has been a very busy first half of the year for the IUIS IEI Committee, with much going on. In March, we held a face-to-face meeting at Rockefeller University, New York. The main goal of this meeting was to provide a critical review of the recent biomedical literature and identify and assess key publications that have reported novel genes that, when mutated, underlie monogenic immune dysregulatory conditions such as recurrent, severe and often-fatal infections, autoinflammatory disorders, autoimmunity, allergic disease and even cancer. Since we published the last update of genes causing inborn errors of immunity (2018), approx. 100 new genes have been reported to affect immunity. After rigorous discussion, the committee agreed on including about 70 of these. Thus, when our next update is published in 2020, we will report >400 genes as molecular defects underlying a vast array of monogenic immunopathologies.

The committee meeting was held at Rockefeller to take advantage of the annual meeting of the Henry Kunkel Society, which this year focussed on “New methods applied to inborn errors of human immunity: immunological and clinical impact”. As such, several of the committee members were presenters at this meeting (Jean-Laurent Casanova, Stuart Tangye), while many others attended.

Members attending our annual face to face meeting in NY, March 2029: L-R – Amos Etzioni, Hans Ochs, Charlotte Cunningham-Rundles, Jose Luis Franco, Jean-Laurent Casanova, Kate Sullivan, Troy Torgeson, Jennifer Puck, Talal Chatila, Stuart Tangye, Christoph Klien, Eric Oksenhendler, Waleed Al Herz; Absent: Capucine Picard, Aziz Bousfiha, Mini Tang, Steve Holland, Tomohiro Morio, Yanick Crow)

This meeting also was an opportunity to offer substantial thanks to several members who have decided to step down from the committee after many years of hard work, dedication and commitment. I would like to take this opportunity to give heartfelt thanks to the following retiring committee members:

  • Hans Ochs (USA)
  • Talal Chatila (USA)
  • Amos Etzioni (Israel)
  • Yanick Crow (UK/France)
  • Mimi Tang (Australia)

This enabled us to invite new members to join the committee. Nominations were sort and then voted. So I now very happy to welcome the following colleagues who were selected by their peers on the committee, and have graciously accepted our invitation to be part of a very dynamic and exciting committee:

  • Isabelle Meyts (Belgium)
  • Anne Puel (France)
  • Mikko Seppanen (Finland)
  • Raz Somech (Israel)
  • Helen Su (USA)

The immediate task for the committee is to finalise a paper detailing the latest update to the list of genes underlying inborn errors of immunity, which we will hopefully have published in the first quarter of 2020. We will next meet formally during the “2nd European Society for Immunodeficiencies (ESID) Focussed Meeting: PID and Malignancy” which will be held in September in Brussels, Belgium. Again, several committee members will be giving key presentations at this major meeting of the field of inborn errors of immunity. The committee will also be represented at the upcoming IUIS Beijing 2019 conference, with Kate Sullivan and Isabelle Meyts being invited participants and speakers.

Another important role of the committee is in educating and training clinicans, registrars, specialists, As such, many of us participate in various teaching courses to enable greater understanding of basic, clinical and translational immunology in general, but also to provide current updates and the latest information relating to developments in the field of inborn errors of immunity. Over the past few months, the following presentations have been given at the indicated meetings, courses, or teaching opportunities:

Aziz Bousfiha

  • 6th ASID congress, Dakar, Senegal, April 2019: Phenotypical Classification of Primary Immunodeficiencies.

Stuart Tangye

  • 47th Annual Scientific Meeting of the Australasian Society of Immunology, Perth, Australia, Dec 2018. Defects in human lymphocyte differentiation in primary human immunodeficiencies. 
  • 27th Annual Henry Kunkel Society Meeting: New methods applied to inborn errors of human immunity: immunological and clinical impact, Rockefeller University, New York, NY, USA. March 2019. Learning human immunology from the study of PIDs. 
  • IDFA (Immune deficiency Foundatin of Australia) Biennielle National Conference, Brisbane, Australia, April 2019. Genetic Testing for primary human immunodeficiencies.
  • International Day of Immunology, Garvan Institute, Sydney, Australia, May 2019. This is how immunology is great.
  • Australasian Society for Clinical Immunology and Allergy (ASCIA) Primary Immunodeficiency Summer School, Sydney, Australia, May 2019. I presented lectures on: (1) Basic science of B-cell disorders; (2) Basic science of combined immunodeficiencies; (3) PIDs associated with hyper-IgE; (4) Basic science of Immune dysregulation

Before I sign off, i would like to thank all of my committee colleagues – former, current and future – for making this a great collective of people working to provide an important service to the community. I am particularly grateful to past chairperson Kate Sullivan who always offers excellent advice, and Jose Luis Franco, who is a wealth of information and guidance. And also our project manager Antonis Pattakos. For those in the north, have a great summer; for those including myself in the southern hemisphere, I wish you warmth. And good health to everyone!

All the best,

Stuart Tangye, PhD

Stuart Tangye, PhD
Ph: +61 2 9295 8455

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