Invariant natural killer T cells in patients with common variable immunodeficiency

Paquin‐Proulx, Dominic; Santos, Bianca A. N.; Carvalho, Karina I.; Toledo-Barros, Myrthes; Oliveira, Ana Karolina Barreto de; Kokron, Cristina Maria; Kalil, Jorge; Moll, Markus; Kallas, Esper G.; Sandberg, Johan K.

doi:10.1016/j.jaci.2014.06.039

Cited by 3 publications

(1 citation statement)

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“…In addition, the distribution of iNKT cell subsets defined by CD4 and CD8 is skewed in CVID, with an increase in the CD4 + and a decrease in the CD8 + subset reported in one cohort ( 28 ). The function of iNKT seems to be relatively preserved in treatment-naïve CVID patients, as only a trend for reduced IFNγ production was seen after stimulation with the model antigen α-Gal–Cer ( 29 ). Increased IFNγ production by iNKT cells was reported for a small number of CVID patients on going IVIg after in vitro expansion ( 30 ).…”

Section: Innate Immune Activationmentioning

confidence: 99%

Persistent Immune Activation in CVID and the Role of IVIg in Its Suppression

Paquin‐Proulx

Sandberg

2014

Front. Immunol.

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Common variable immunodeficiency (CVID) is one of the most common and clinically important primary immune deficiencies. CVID patients have poor humoral immunity, resulting in recurrent infections of the gastrointestinal and upper respiratory tracts, as well as increased incidence of some forms of cancers and autoimmune diseases. The treatment for CVID is IgG replacement, often given as intravenous immunoglobulins (IVIg). IVIg consists of monomeric IgG purified from pooled plasma from healthy donors and is used to treat an increasing number of conditions including autoimmune diseases. In the case of CVID, IVIg has mainly been seen as reconstitution therapy, providing patients with pathogen-specific antibodies. Recent evidence shows that IVIg has diverse effects on the immune system of CVID patients, and one important component is that IVIg alleviates the state of chronic immune activation. In this review, we will discuss causes and consequences of persistent immune activation in CVID, possible underlying mechanisms for how IVIg treatment reduces immune activation, and implications for our understanding of primary as well as acquired immune deficiencies.

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Section: Innate Immune Activationmentioning

confidence: 99%