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The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema

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Abstract

Hereditary angioedema (HAE) is a rare autosomic-dominant disorder characterized by a deficiency of C1 esterase inhibitor which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways that are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis during attack and remission state and n = 19 healthy subjects. The samples were tested for a panel of IL (Interleukin)-17-type cytokines (IL-1β, IL-6, IL-10, granulocyte–macrophage colony stimulating factor (GM-CSF), IL-17, IL-21, IL-22, IL-23) and transforming growth factor-beta (TGF-β) subtypes. Data indicate that there are variations of cytokine levels in HAE subjects comparing the condition during the crisis respect to the value in the remission phase, in particular type 17 signature cytokines are increased, whereas IL-23 is unmodified and TGF-β3 is significantly reduced. When comparing healthy and HAE subjects in the remission state, we found a significant difference for IL-17, GM-CSF, IL-21, TGF-β1 and TGF-β2 cytokines. These results confirm and extend our previous findings indicating that in HAE there is operating an inflammatory activation process, which involves also T helper 17 (Th17) cytokines and TGF-β isoforms, associated with localized angioedema attacks and characterized by elevated bradykinin levels.

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Funding

This study was funded by Hospital funds.

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Authors and Affiliations

  1. Patologia Clinica Presidio Ospedaliero V. Cervello, Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy

    Francesco Arcoleo & Mariangela Lo Pizzo

  2. Dipartimento di Biopatologia e Biotecnologie Mediche (DIBIMED), University of Palermo, Palermo, Italy

    Gabriella Misiano, Salvatore Milano & Giuseppina Colonna Romano

  3. Dipartimento Scienze Economiche, Aziendali e Statistiche, University of Palermo, Palermo, Italy

    Vito Muggeo

  4. Dipartimento Immunologia Allergologia, Istituto Medico Europeo (ISME), Palermo, Italy

    Enrico Cillari

Authors
  1. Francesco Arcoleo
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  2. Mariangela Lo Pizzo
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  3. Gabriella Misiano
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  4. Salvatore Milano
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  5. Giuseppina Colonna Romano
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  6. Vito Muggeo
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  7. Enrico Cillari
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Correspondence to Francesco Arcoleo.

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The authors declare that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

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Informed consent was obtained from all individual participants included in the study.

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Arcoleo, F., Lo Pizzo, M., Misiano, G. et al. The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema. Clin Exp Med 18, 355–361 (2018). https://doi.org/10.1007/s10238-018-0499-0

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  • DOI: https://doi.org/10.1007/s10238-018-0499-0

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