[HTML][HTML] Common variable immunodeficiency-an update

U Salzer, K Warnatz, HH Peter - Arthritis research & therapy, 2012 - Springer
Common variable immunodeficiency (CVID) describes a heterogeneous subset of
hypogammaglobulinemias of unknown etiology. Typically, patients present with recurrent …

[HTML][HTML] Is dosing of therapeutic immunoglobulins optimal? A review of a three-decade long debate in Europe

J Kerr, I Quinti, M Eibl, H Chapel, PJ Späth… - Frontiers in …, 2014 - frontiersin.org
The consumption of immunoglobulins (Ig) is increasing due to better recognition of antibody
deficiencies, an aging population, and new indications. This review aims to examine the …

The crossroads of autoimmunity and immunodeficiency: Lessons from polygenic traits and monogenic defects

B Grimbacher, K Warnatz, PFK Yong… - Journal of Allergy and …, 2016 - Elsevier
Autoimmune and immunodeficiency diseases are outcomes of a dysfunctional immune
system and represent 2 sides of the same coin. Multiple single-gene defects have been …

Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency

B Grimbacher, A Hutloff, M Schlesier, E Glocker… - Nature …, 2003 - nature.com
No genetic defect is known to cause common variable immunodeficiency (CVID), a
heterogeneous human disorder leading to adult-onset panhypogammaglobulinemia. In a …

The EUROclass trial: defining subgroups in common variable immunodeficiency

C Wehr, T Kivioja, C Schmitt, B Ferry… - Blood, The Journal …, 2008 - ashpublications.org
The heterogeneity of common variable immunodeficiency (CVID) calls for a classification
addressing pathogenic mechanisms as well as clinical relevance. This European …

Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans

U Salzer, HM Chapel, ADB Webster… - Nature …, 2005 - nature.com
The functional interaction of BAFF and APRIL with TNF receptor superfamily members
BAFFR, TACI and BCMA is crucial for development and maintenance of humoral immunity in …

Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist

JH Korn, M Mayes, M Matucci Cerinic… - Arthritis & …, 2004 - Wiley Online Library
Objective Recurrent digital ulcers are a manifestation of vascular disease in patients with
systemic sclerosis (SSc; scleroderma) and lead to pain, impaired function, and tissue loss …

Human Immunoglobulin M Memory B Cells Controlling Streptococcus pneumoniae Infections Are Generated in the Spleen

S Kruetzmann, MM Rosado, H Weber… - The Journal of …, 2003 - rupress.org
Splenectomized and asplenic patients have a high incidence of infections by encapsulated
bacteria and do not respond to polysaccharide vaccines. To understand whether the …

Severe deficiency of switched memory B cells (CD27+IgMIgD) in subgroups of patients with common variable immunodeficiency: a new approach to classify a …

K Warnatz, A Denz, R Drager, M Braun… - Blood, The Journal …, 2002 - ashpublications.org
Hypogammaglobulinemia is the hallmark of common variable immunodeficiency (CVID)
syndrome, a heterogeneous disorder predisposing patients to recurrent bacterial infections …

B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans

K Warnatz, U Salzer, M Rizzi… - Proceedings of the …, 2009 - National Acad Sciences
B-cell survival depends on signals induced by B-cell activating factor (BAFF) binding to its
receptor (BAFF-R). In mice, mutations in BAFF or BAFF-R cause B-cell lymphopenia and …