Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis

Grzegorz Porębski; Jacek Gocki; Aldona Juchacz; Aleksandra Kucharczyk; Tomasz Matuszewski; Katarzyna Olejniczak; Małgorzata Sokołowska; Marcin Stobiecki; Ewa Trębas-Pietraś; Krystyna Obtułowicz

doi:10.5114/pja.2018.76679

eISSN: 2391-6052
ISSN: 2353-3854

Alergologia Polska - Polish Journal of Allergology

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abstract:

Guidelines/recommendations

Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis

Grzegorz Porębski

,

Jacek Gocki

,

Aldona Juchacz

,

Aleksandra Kucharczyk

,

Tomasz Matuszewski

,

Katarzyna Olejniczak

,

Małgorzata Sokołowska

,

Marcin Stobiecki

,

Ewa Trębas-Pietraś

,

Krystyna Obtułowicz

Alergologia Polska – Polish Journal of Allergology 2018; 5, 2: 98–108

DOI: https://doi.org/10.5114/pja.2018.76679

Online publish date: 2018/06/27

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AMA

Porębski G, Gocki J, Juchacz A, et al. Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis. Alergologia Polska - Polish Journal of Allergology. 2018;5(2):98-108. doi:10.5114/pja.2018.76679.

APA

Porębski, G., Gocki, J., Juchacz, A., Kucharczyk, A., Matuszewski, T.,  & Olejniczak, K. et al. (2018). Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis. Alergologia Polska - Polish Journal of Allergology, 5(2), 98-108. https://doi.org/10.5114/pja.2018.76679

Chicago

Porębski, Grzegorz, Jacek Gocki, Aldona Juchacz, Aleksandra Kucharczyk, Tomasz Matuszewski, Katarzyna Olejniczak,  and Małgorzata Sokołowska et al. 2018. "Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis". Alergologia Polska - Polish Journal of Allergology 5 (2): 98-108. doi:10.5114/pja.2018.76679.

Harvard

Porębski, G., Gocki, J., Juchacz, A., Kucharczyk, A., Matuszewski, T., Olejniczak, K., Sokołowska, M., Stobiecki, M., Trębas-Pietraś, E.,  and Obtułowicz, K. (2018). Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis. Alergologia Polska - Polish Journal of Allergology, 5(2), pp.98-108. https://doi.org/10.5114/pja.2018.76679

MLA

Porębski, Grzegorz et al. "Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis." Alergologia Polska - Polish Journal of Allergology, vol. 5, no. 2, 2018, pp. 98-108. doi:10.5114/pja.2018.76679.

Vancouver

Porębski G, Gocki J, Juchacz A, Kucharczyk A, Matuszewski T, Olejniczak K et al. Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis. Alergologia Polska - Polish Journal of Allergology. 2018;5(2):98-108. doi:10.5114/pja.2018.76679.

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Hereditary angioedema (HAE), resulting from inherited deficiency of C1 inhibitor, is a rare but disabling and life-threatening disease. An overshooting local increase in bradykinin concentration induces angioedema attacks, which manifest as acute abdominal pain, and facial, laryngeal or peripheral swellings. Health care providers are often not aware of appropriate differential diagnosis and therapy of this rare disease, which frequently bears resemblance to other disorders. For this reason we convened the working group of the HAE Section members experienced in HAE management to develop recommendations for Polish clinical decision makers. The document was initiated at the working meeting and then circulated for further suggestions. The independent review board was requested to review the document and provide feedback. This first part of the consensus statement covers clinical symptoms, differential diagnosis, classification, and pathophysiologic background of HAE, including new genetic findings.

keywords:

Management of hereditary angioedema with C1 inhibitor-deficiency – consensus statement of the HAE Section of the Polish Society of Allergology. Part I: classification, pathophysiology, clinical symptoms, and diagnosis

Grzegorz Porębski , Jacek Gocki , Aldona Juchacz , Aleksandra Kucharczyk , Tomasz Matuszewski , Katarzyna Olejniczak , Małgorzata Sokołowska , Marcin Stobiecki , Ewa Trębas-Pietraś , Krystyna Obtułowicz

hereditary angioedema, C1-inhibitor, diagnosis, classification, recommendations

Grzegorz Porębski

,

Jacek Gocki

,

Aldona Juchacz

,

Aleksandra Kucharczyk

,

Tomasz Matuszewski

,

Katarzyna Olejniczak

,

Małgorzata Sokołowska

,

Marcin Stobiecki

,

Ewa Trębas-Pietraś

,

Krystyna Obtułowicz