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Primary Immunodeficiency Expert Committee

 

HISTORY

The Primary Immunodeficiency Expert Committee was originally set up under the auspices of the World Health Organisation in 1973, to describe and classify the types of primary immune defects affecting humans.
The classification of those diseases recognised at the time was published in a report (Cooper MD et al NEJM 288 966-967) and revised every two years subsequently.
However in the 1990s the WHO made a policy decision to concentrate on more common diseases, so the IUIS agreed to take on the remit of this committee; this was formalised at the 54th IUIS Council meeting in Tapei in 2008.

 

MISSION STATEMENT

  • To provide an up-to-date classification of all primary immunodeficiency diseases (PIDs)
  • To assist with the identification, diagnosis and management of patients with these uncommon conditions
  • To support diagnostic and therapeutic guidelines developed by national societies and others, to assist healthcare providers
  • To promote awareness, diagnosis and treatment of PIDs in all regions of the world
  • To produce ad hoc reports on any aspect of PIDs, to assist in the welfare of patients with these conditions

 

CLASSIFICATION TABLES

An up-to-date report on an ever-increasing list of primary immunodeficiencies is produced biennially.  The latest version “Classification of Primary Immunodeficiency Diseases by the International Union of Immunological Societies (IUIS) Expert Committee on Primary Immunodeficiency 2011“ is published in Frontiers of Immunology: Primary Immunodeficienceis, the IUIS Journal www.frontiersin.org/primary_immunodeficiencies 
The process of considering reports of new primary immune deficiencies, in order to classify them appropriately, continues at biennial meetings held in different continents. The most recent meeting was held in New York in June 2011 and the Classification Tables can be downloaded from this link for worldwide access.

Since there are now over 180 primary immune deficiencies, with about 20 new ones per year, the report provides important definitions for clinical management and standardised research, as well as for patients. Readers of the report include clinicians who use this document in their daily practice in many countries and scientists investigating the immunologically mechanisms that prevent infectious diseases in humans.
Many national and international healthcare providers use the document for the basis of funding therapies and professional medical bodies have based their diagnostic guidelines on this classification. There are now templates for use by national professional bodies contemplating new guidance and these can be downloaded here.