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Blood, 15 April 2002, Vol. 99, No. 8, pp. 2694-2702
REVIEW ARTICLE
Autoimmunity in human primary immunodeficiency diseases
Peter D. Arkwright,
Mario Abinun, and
Andrew J. Cant
From the Academic Unit of Child Health, University of
Manchester, St Mary's Hospital, Manchester, and the Paediatric
Immunology and Infectious Diseases Unit, Newcastle General Hospital,
Newcastle upon Tyne, United Kingdom.
Human primary immunodeficiency diseases are experiments of nature
characterized by an increased susceptibility to infection. In many
cases, they are also associated with troublesome and sometimes life-threatening autoimmune complications. In the past few years, great
strides have been made in understanding the molecular basis of primary
immunodeficiencies, and this had led to more focused and successful
treatment. This review has 3 aims: (1) to highlight the variety of
autoimmune phenomena associated with human primary immunodeficiency
diseases; (2) to explore how primary immunodeficiencies predispose
patients to autoimmune phenomena triggered by opportunistic infections;
and (3) to consider the rationale for the current treatment strategies
for autoimmune phenomena, specifically in relation to primary
immunodeficiency diseases. Reviewing recent advances in our
understanding of the small subgroup of patients with defined causes for
their autoimmunity may lead to the development of more effective
treatment strategies for idiopathic human autoimmune diseases.
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