共 74 条
Common variable immunodeficiency - an update
被引:126
作者:

Salzer, Ulrich
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机构:
Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany
Univ Med Ctr Freiburg, Dept Rheumatol, D-79106 Freiburg, Germany Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany

Warnatz, Klaus
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机构:
Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany
Univ Med Ctr Freiburg, Dept Rheumatol, D-79106 Freiburg, Germany Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany

Peter, Hans Hartmut
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机构:
Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany
Univ Med Ctr Freiburg, Dept Rheumatol, D-79106 Freiburg, Germany Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany
机构:
[1] Univ Med Ctr Freiburg, Ctr Chron Immunodeficiency, D-79108 Freiburg, Germany
[2] Univ Med Ctr Freiburg, Dept Rheumatol, D-79106 Freiburg, Germany
关键词:
MEMORY B-CELLS;
PRIMARY ANTIBODY DEFICIENCIES;
SELECTIVE IGA DEFICIENCY;
REGULATORY T-CELLS;
IMMUNOLOGICAL FEATURES;
SUSCEPTIBILITY LOCUS;
IMMUNOGLOBULIN;
LINKAGE;
ASSOCIATION;
LYMPHOCYTES;
D O I:
10.1186/ar4032
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Common variable immunodeficiency (CVID) describes a heterogeneous subset of hypogammaglobulinemias of unknown etiology. Typically, patients present with recurrent bacterial infections of the respiratory and gastrointestinal tract. A significant proportion of CVID patients develops additional autoimmune, inflammatory or lymphoproliferative complications. CVID is the most frequent symptomatic primary immunodeficiency encountered in adults. Informative monogenetic defects have been found in single patients and families but in most cases the pathogenesis is still elusive. Numerous immunological studies have demonstrated phenotypic and functional abnormalities of T cells, B cells and antigen-presenting cells. A hallmark is the impaired memory B-cell formation that has been taken advantage of for classifying CVID patients. Clinical multi-center studies have demonstrated a correlation between immunological markers and clinical presentation. Long-term outcome is significantly influenced by delay of diagnosis and treatment and the presence of chronic inflammatory complications. While immunoglobulin replacement therapy plus antibiotics can control infections in most cases, patients with non-infectious inflammatory complications such as granulomatous inflammation, interstitial lung disease, inflammatory bowel disease, lymphoproliferation and developing malignancies still represent a therapeutic challenge. In this review we provide a systematic overview of the immunological, clinical, diagnostic and therapeutic aspects of CVID and highlight recent developments in these fields.
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共 74 条
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Zandieh, F
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Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran

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Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran

Ghazi, BM
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Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran

Mahmoudi, M
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Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran Univ Tehran Med Sci, Childrens Med Ctr, Dept Clin Immunol, Immunol Asthma & Allergy Res Inst, Tehran, Iran

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Mouillot, Gael
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Gerard, Laurence
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Carmagnat, Maryvonnick
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Rabian, Claire
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Pasquali, Jean-Louis
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机构:
Hop Univ Strasbourg, Dept Clin Immunol & Internal Med, Strasbourg, France
Univ Strasbourg, CNRS, UPR9021, Strasbourg, France Hop Univ Strasbourg, Dept Clin Immunol & Internal Med, Strasbourg, France

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