Autoimmunity in common variable immunodeficiency: epidemiology, pathophysiology and management

被引：61

作者：

Azizi, Gholamreza ^{[1
,2
,3
]}

Abolhassani, Hassan ^{[2
,4
]}

Asgardoon, Mohammad Hosein ^{[2
,5
]}

Alinia, Tina ^{[2
]}

Yazdani, Reza ^{[6
]}

Mohammadi, Javad ^{[7
]}

Rezaei, Nima ^{[2
]}

Ochs, Hans D. ^{[8
,9
]}

Aghamohammadi, Asghar ^{[2
,3
]}

机构：

[1] Alborz Univ Med Sci, Imam Hassan Mojtaba Hosp, Dept Lab Med, Karaj, Iran

[2] Univ Tehran Med Sci, Res Ctr Immunodeficiencies, Pediat Ctr Excellence, Childrens Med Ctr, Tehran, Iran

[3] USERN, PIDNet, Tehran, Iran

[4] Karolinska Inst, Karolinska Univ Hosp Huddinge, Div Clin Immunol, Dept Lab Med, Stockholm, Sweden

[5] Univ Tehran Med Sci, Students Sci Res Ctr, Iranian Student Soc Immunodeficiencies, Tehran, Iran

[6] Isfahan Univ Med Sci, Sch Med, Dept Immunol, Esfahan, Iran

[7] Univ Tehran, Fac New Sci & Technol, Dept Biomed Engn, Tehran, Iran

[8] Univ Washington, Seattle Childrens Res Inst, Seattle, WA 98195 USA

[9] Univ Washington, Dept Pediat, Seattle, WA 98195 USA

来源：

EXPERT REVIEW OF CLINICAL IMMUNOLOGY | 2017年 / 13卷 / 02期

关键词：

Common variable immunodeficiency; autoimmunity; autoantibody; management; primary antibody deficiency; SYSTEMIC-LUPUS-ERYTHEMATOSUS; IDIOPATHIC THROMBOCYTOPENIC PURPURA; IMMUNOGLOBULIN REPLACEMENT THERAPY; INTERSTITIAL LUNG-DISEASE; X-LINKED AGAMMAGLOBULINEMIA; PRIMARY ANTIBODY DEFICIENCY; DENDRITIC CELL-FUNCTION; BONE-MARROW EXAMINATION; AUTOREACTIVE B-CELLS; TOLL-LIKE RECEPTORS;

D O I：

10.1080/1744666X.2016.1224664

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Introduction: Common variable immunodeficiency (CVID) comprises a large heterogeneous group of patients with primary antibody deficiency.Areas covered: The affected patients are characterized by increased susceptibility to infections and low levels of serum immunoglobulin. However, enteropathy, granulomatous organ infiltrates, malignancy, inflammatory and autoimmune conditions are also prevalent. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and creates difficulties in the management of autoimmune complications affecting these patients.Expert commentary: The management of autoimmunity in patients with CVID requires special considerations because dysregulation and dysfunctions of the immune system along with persistent inflammation impair the process of diagnosis and treatment.

引用

页码：101 / 115

页数：15

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