[Common variable immunodeficiency: a clinical challenge].

Abstract:

:Common variable immunodeficiency (CVID) represents the most common clinically relevant form of primary immunodeficiency. This heterogeneous antibody deficiency syndrome is characterized not only by susceptibility to bacterial respiratory tract infections but displays additional signs of immune dysregulation, such as autoimmunity, chronic inflammation and lymphoproliferation in more than 30 % of the patients. Due to poor awareness the diagnosis is often delayed by 4-6 years. A close collaboration in patient care with a center specialized in primary immunodeficiency is recommended. Regular follow-up visits include assessment of adequate immunoglobulin replacement therapy and screening for manifestation of secondary complications. Regular substitution with intravenous or subcutaneous immunoglobulins has more or less normalized life expectancy of patients with isolated susceptibility to bacterial infections. Therefore, the current core task in the management of CVID patients is the elaboration of more effective and safer forms of prophylaxis and treatment of sequelae of immune dysregulation in the lungs, intestines and liver of affected patients.

journal_name

Z Rheumatol

authors

Warnatz K,Goldacker S

doi

10.1007/s00393-013-1162-5

subject

Has Abstract

pub_date

2013-09-01 00:00:00

pages

653-60, 662

issue

7

eissn

0340-1855

issn

1435-1250

journal_volume

72

pub_type

杂志文章
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    doi:

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  • [Tocilizumab. What comes after TNF-blockers in clinical routine?].

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  • [Blastocystis hominis as a rare arthritogenic pathogen. A case report].

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  • [German 2012 guidelines for the sequential medical treatment of rheumatoid arthritis. Adapted EULAR recommendations and updated treatment algorithm].

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