Autoantibodies against BAFF, APRIL or IL21 - an alternative pathogenesis for antibody-deficienciesReportar como inadecuado

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BMC Immunology

, 18:34

Cellular immunology and immune regulation


BackgroundThe ability of anti-cytokine antibodies to play a disease-causing role in the pathogenesis of immunodeficiencies is widely accepted. The aim of this study was to investigate whether autoantibodies against BAFF important B cell survival signal, APRIL important plasma cell survival signal, or Interleukin-21 important cytokine for immunoglobulin class switch present an alternative mechanism for the development of the following primary antibody deficiencies PADs: common variable immune deficiency CVID or selective IgA deficiency sIgAD.

ResultsTwo hundred thirty-two sera from patients with PADs were screened for autoantibodies against cytokines by ELISA. Statistical data analysis yielded a significant difference p < 0.01 between the healthy donor sera and both PAD cohorts. The analysis was deepened by subdividing the patient collective into groups with distinct B cell phenotypes but no significant differences were found. For selected sera with notable high ELISA-read outs functional analysis ensued. Anti-BAFF and anti-APRIL antibodies were further examined by a B cell survival assay, whilst the functional relevance of putative anti-IL-21 autoantibodies was investigated by means of a STAT3 phosphorylation assay. However, the results of these experiments revealed no discernible functional effect.

ConclusionWhilst statistical analysis of ELISA results showed significant differences between patients and healthy controls, in our set of patients functional tests yielded no evidence for an involvement of autoantibodies against BAFF, APRIL, or IL-21 in the pathogenesis of CVID or sIgAD.

KeywordsAutoimmunity Autoantibodies Cytokines Primary antibody deficiency Common variable immunodeficiency Selective IgA deficiency BAFF APRIL IL-21 AbbreviationsAPRILA proliferation inducing ligand

APS-IAutoimmune polyendocrinopathy I

BAFFB cell activating factor

CMCChronic mucocutaneous candidiasis

CVIDCommon variable immunodeficiency

IFN-gammaInterferon gamma

IL-21Interleukin 21

sIgADselective IgA deficiency

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Autor: Marian-Christopher Pott - Natalie Frede - Jennifer Wanders - Lennart Hammarström - Erik-Oliver Glocker - Cristina Glocker -


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