Low ficolin-2 levels in common variable immunodeficiency patients with bronchiectasis

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METZGER M.L. MICHELFELDER I. GOLDACKER S. MELKAOUI K. LITZMAN Jiří GUZMAN D. GRIMBACHER B. SALZER U.

Rok publikování 2015
Druh Článek v odborném periodiku
Časopis / Zdroj Clinical and Experimental Immunology
Fakulta / Pracoviště MU

Lékařská fakulta

Citace
Doi http://dx.doi.org/10.1111/cei.12459
Obor Imunologie
Klíčová slova complement; CVID; ficolin-2; ficolin-3; lectin pathway
Popis Common variable immunodeficiency (CVID) encompasses a heterogeneous group of antibody deficiencies characterized by susceptibility to recurrent infections and sequelae, including bronchiectasis. We investigated the relevance of the lectin complement pathway in CVID patients by analysing ficolin-2 and ficolin-3 serum levels and genotyping single nucleotide polymorphisms (SNPs) in the FCN2 and FCN3 genes. Our results show that ficolin-2 levels in CVID patients are significantly lower (P<00001) than in controls. The lowest ficolin-2 levels are found in CVID patients with bronchiectasis (P=00004) and autoimmunity (P=004). Although serum levels of ficolin-3 were similar in CVID patients and controls, CVID patients with bronchiectasis again showed lower levels when compared to controls (P=00001). Analysis of single nucleotide polymorphisms in the FCN2 gene confirmed known influences on ficolin-2 serum levels, but did not support a genetic basis for the observed ficolin-2 deficiency in CVID. We found that CVID patients with bronchiectasis have very low levels of ficolin-2. The reason for the deficiency of ficolin-2 in CVID and any possible causal relationship is currently unknown. However, as bronchiectasis is a very important factor for morbidity and mortality in CVID, ficolin-2 could also serve as biomarker for monitoring disease complications such as bronchiectasis.
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