International Consensus Document (ICON): Common Variable Immunodeficiency Disorders

Abstract

The International Collaboration in Asthma, Allergy and Immunology initiated an international coalition among the American Academy of Allergy, Asthma & Immunology; the European Academy of Allergy and Clinical Immunology; the World Allergy Organization; and the American College of Allergy, Asthma & Immunology on common variable immunodeficiency. An author group was formed and then divided into individual committees. Within the committee, teams of authors were subgrouped to generate content for specific sections of the document. Content was derived from literature searches, relevant published guidelines, and clinical experience. After a draft of the document was assembled, it was collectively reviewed and revised by the authors. Where evidence was lacking or conflicting, the information presented represents the consensus expert opinion of the group. The full document was then independently reviewed by 5 international experts in the field, none of whom was among the authors of the original. The comments of these reviewers were incorporated before submission for publication.

The immunophenotypic fingerprint of patients with primary antibody deficiencies is partially present in their asymptomatic first-degree relatives

“…2 Common variable immunodeficiency (CVID) is one of the most common PAD and is a clinically and immunologically heterogeneous disorder. 2,3 Indeed, the definition of CVID is a topic of ongoing debate. The term CVID was introduced in 1971 to distinguish less well-defined PAD from those with a consistent phenotype and inheritance.…”

“…7 However, not all practitioners agree on the obligatory decrease in IgA. 3 In 2016, an international consensus statement on CVID proposed less stringent diagnostic criteria, closely resembling the ESID/PAGID 1999 criteria and not including a reduction in memory B cells. 3 CVID patients have an increased susceptibility to infections, predominantly of the respiratory tract.…”

“…3 In 2016, an international consensus statement on CVID proposed less stringent diagnostic criteria, closely resembling the ESID/PAGID 1999 criteria and not including a reduction in memory B cells. 3 CVID patients have an increased susceptibility to infections, predominantly of the respiratory tract. 3,8 Moreover, they are prone to developing non-infectious complications such as autoimmunity, polyclonal lymphoproliferation, and malignancies.…”

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“…Folglich ist die Anzahl der Memory-Zellen nach einem durchgemachten Infekt reduziert oder fehlt ganz, was zu einer schnellen Reinfektion mit dem identischen Erreger führen kann. Knochenmarkuntersuchungen zeigten in 94 % der CVID-Patienten eine Depletion der Plasmazellen, die direkt mit den IgG-Spiegeln im Blut korreliert [1]. Als Reaktion auf die fehlende oder fehlerhafte Aktivierung der B-Zellen zeigt sich eine Überproduktion der unreifen B-Zellen im Knochenmark und lymphatischen Gewebe, eine sogenannte Lymphoproliferation, welche in 20 % der Patienten klinisch zur zervikalen, mediastinalen und abdominellen Lymphadenopathie, Splenomegalie und Granulombildung führt [1].…”

“…Knochenmarkuntersuchungen zeigten in 94 % der CVID-Patienten eine Depletion der Plasmazellen, die direkt mit den IgG-Spiegeln im Blut korreliert [1]. Als Reaktion auf die fehlende oder fehlerhafte Aktivierung der B-Zellen zeigt sich eine Überproduktion der unreifen B-Zellen im Knochenmark und lymphatischen Gewebe, eine sogenannte Lymphoproliferation, welche in 20 % der Patienten klinisch zur zervikalen, mediastinalen und abdominellen Lymphadenopathie, Splenomegalie und Granulombildung führt [1]. Die Lymphoproliferation ist auch nach Therapiebeginn mit Immunglobulinsubstitution nicht reversibel und kann mit der Zeit zu lymphoproliferativen Erkrankungen (u.a.…”