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Clinical WP - Newsletter August 2020

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Welcome to my last newsletter as Chair of Clinical Working Party. It has been a privilege to lead this group.

The guidelines writing continues:

The Complement Disorders guidelines have been published in the Journal of Clinical Immunology1. A consensus statement on malignancy and Primary immunodeficiency has also been published2, and more recently, in a collaboration between the Clinical Working Party, Inborn Errors Working Party and Registry Working Party, a publication on outcome of patients with LRBA deficiency treated with various modalities3. This is a particularly important publication as it shows the value of the ESID Registry in answering questions about different diseases – in this case related to treatment – and the collaboration of IEWP and CWP to compare transplant and non-transplant outcomes. I hope that many further projects like this may be realized in the future.

  Current and emerging guidelines:

  • Vaccination of the immunocompromised (Filomeen Haerynck + Andy Gennery: [email protected]    ) – writing committee convened, draft prepared and currently in internal revision
  • Consensus statement on diagnosis and management of CGD (Siobhan Burns + Andy Gennery:    ) – writing in progress
  • Consensus statement on diagnosis and management of GLILD in CVID (Klaus Warnatz: )
  • Thymic Disorders (Aisling Flinn, Graham Davies, Andy Gennery)

  

Additional upcoming topics include:

Secondary Immunodeficiency

Siobhan Burns is leading a project on outcome of patients with XIAP deficiency with or without HSCT – with ESID Registry and the collaboration of IEWP.

Andy Gennery is leading a project on Investigating Health Status And Quality Of Life In Patients With Autosomal Dominant Hyper IgE Syndrome – with ESID Registry.

A further project is in progress in conjunction with the IEWP and Registry WP to construct a data set for follow up of patients with idiopathic CD4+ lymphocytopenia, detected by newborn screening.

 

Meetings

As you know, ESID will be ‘virtual’ this year, but we will still have a CWP business meeting as part of this. We are planning a meeting on Secondary Immunodeficiencies – probably next Spring (2021), but time and venue have yet to be finalized. We will have the 4th Thymic Workshop in November this year – also virtual (contact for details).

 

ESID Elections

I will be stepping down in September and would like to thank the steering committee for their support over the last 4 years – we have 4 excellent candidates standing for the position of Chair of CWP so please vote for one of them. 

  1. Raffaele Badolato
  2. Siobhan Burns
  3. Stephen Jolles
  4. Benedicte Neven

 

Best wishes, 

Andy Gennery 

1. Brodszki N, Frazer-Abel A, Grumach AS, et al. European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA). Complement Guideline: Deficiencies, Diagnosis and Management. J Clin Immunol 2020;40:576-591

2. Bomken S, van der Werff Ten Bosch J, Attarbaschi A, et al: on behalf of The Clinical and Inborn Errors Working Parties of the European Society for Immunodeficiencies (ESID) and European Society for Blood and Marrow Transplantation (EBMT), the European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune diseases (RITA), The Host Variation Task Force of the International Berlin-Frankfurt-Munster (iBFM) Study Group and the European Intergroup Collaboration for Childhood non-Hodgkin Lymphoma (EICNHL). Current understanding and future research priorities in malignancy associated with primary immunodeficiency and DNA repair disorders: The perspective of an interdisciplinary working group. Front Immunol 2018;9:2192.

3. Tesch VK, Abolhassani H, Shadur B, et al for the Inborn Errors, Clinical and Registry Working Parties of the European Society for Blood and Marrow Transplantation (EBMT) and the European Society of Immunodeficiencies (ESID). Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities evaluated by the immune deficiency and dysregulation activity (IDDA) score. J Allergy Clin Immunol