Foreword
Primary immunodeficiency diseases are a group
of serious but little known disorders in which
immune system malfunction causes increased susceptibility
to infection, autoimmune diseases, and malignancy.
This is a group of more than 70 different diseases,
many of which are inherited. Together they affect
approximately 500,000 individuals in the United
States; of these, between 5,000 and 10,000 people,
many of whom are children, are severely affected
by serious, recurrent, and often life-threatening
infections.
Supported researchers use the most modern methods
available to identify the genetic defects and
other causes of disease, and rapidly apply these
results to the development of improved diagnostic
procedures as well as better treatments to extend
and improve the lives of affected individuals.
This article provides an overview of the primary
immunodeficiency diseases, the significant accomplishments
of NIAID-supported investigators in characterizing
these diseases and their causes and in developing
new therapies, and the prospects for the future
when it may become possible to correct the abnormalities
and cure these diseases.