Abstract：
:Coexisting morbidities in CVID include bronchiectasis, autoimmunity and malignancies. The incidence of autoimmune disease in CVID patients may approach 20% of cases. The most common autoimmune disease found in CVID patients is autoimmune cytopenia, but rheumatoid arthritis, lupus, and now primary biliary cirrhosis have also been reported. The coexistence of immunodeficiency and autoimmunity appears paradoxical, since one represents a hypoimmune state and the other a hyperimmune state. However, this paradox may not actually be all that implausible due to the complex nature of immune cells, signaling pathways and their interactions. The cellular alterations in combined variable immunodeficiency include a range of T and B cell abnormalities. Selective immune derangements found in CVID include a downregulation of regulatory T cells (Treg cells), accelerated T cell apoptosis, abnormal cytokine production secondary to cytokine gene polymorphisms and increased autoreactive B cell production. The impact of these abnormalities on T and B cell interaction may not only explain the immunodeficiency but also the development of autoimmunity in select groups of patients with CVID. The variability in the clinical manifestations of CVID as a result of this immune interaction suggests that CVID is not one disease but many. This is important because it follows that the treatment of CVID may not always be the same, but may need to be directed specifically towards each individual patient.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Xiao X,Miao Q,Chang C,Gershwin ME,Ma Xdoi
10.1016/j.autrev.2014.04.006subject
Has Abstractpub_date
2014-08-01 00:00:00pages
858-64issue
8eissn
1568-9972issn
1873-0183pii
S1568-9972(14)00119-0journal_volume
13pub_type
杂志文章,评审abstract：:Juvenile idiopathic arthritis (JIA), the most common chronic rheumatic disease of childhood, is a clinically heterogeneous group of disorders characterized by chronic inflammatory arthritis. JIA is considered to be an autoimmune disease, which is a result of immune reaction caused or triggered by environmental factors...
journal_title：Autoimmunity reviews
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
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journal_title：Autoimmunity reviews
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2017-09-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章
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更新日期：2020-02-01 00:00:00
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journal_title：Autoimmunity reviews
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doi：10.1016/j.autrev.2012.07.013
更新日期：2012-11-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2011-01-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2017-04-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2009.03.008
更新日期：2009-12-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2012.08.008
更新日期：2013-02-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.12.009
更新日期：2011-05-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.11.026
更新日期：2012-08-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2008.07.022
更新日期：2008-10-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2009.11.010
更新日期：2010-03-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2014.01.022
更新日期：2014-04-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 社论
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更新日期：2017-10-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2017-11-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2018-10-01 00:00:00
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journal_title：Autoimmunity reviews
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2018-07-01 00:00:00
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2004-01-01 00:00:00
abstract：:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCA. Although these autoantibodies were initially used to classify pauci-immune vasculitis, increasing clinical and experimen...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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更新日期：2016-07-01 00:00:00
abstract：:The central function of the immune system is to protect the host from environmental agents such as microbes or chemicals, thereby preserving the integrity of the body, and preventing the onset of illness and infection. Moreover, the immune system is constantly challenged to discriminate self vs. non-self and mediate t...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2016.12.011
更新日期：2017-02-01 00:00:00
abstract：:The classical view of B cells in the biology of autoimmune responses to infectious and self-antigens (Ag) that they promote immunity primarily by producing antibodies (Ab) is far from being complete. Indeed, studies over the last decade suggest that B cells have extraordinarily diverse functions within the immune syst...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2004.07.035
更新日期：2004-11-01 00:00:00
