Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis

Grzegorz Porębski; Aleksandra Kucharczyk; Tomasz Matuszewski; Hanna Danielewicz; Jacek Gocki; Katarzyna Grzela; Aldona Juchacz; Marcin Kurowski; Krzysztof Kuziemski; Mateusz Łukaszyk; Małgorzata Sokołowska; Marcin Stobiecki; Ewa Trębas-Pietraś; Marta Tykwińska; Ewa Czarnobilska; Marcin Moniuszko; Krystyna Obtułowicz

doi:10.5114/pja.2025.153716

eISSN: 2391-6052
ISSN: 2353-3854

Alergologia Polska - Polish Journal of Allergology

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Guidelines/recommendations

Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis

Grzegorz Porębski

¹

,

Aleksandra Kucharczyk

²

,

Tomasz Matuszewski

²

,

Hanna Danielewicz

³

,

Jacek Gocki

⁴

,

Katarzyna Grzela

⁵

,

Aldona Juchacz

⁶

,

Marcin Kurowski

⁷

,

Krzysztof Kuziemski

⁸

,

Mateusz Łukaszyk

⁹

,

Małgorzata Sokołowska

¹⁰

,

Marcin Stobiecki

¹

,

Ewa Trębas-Pietraś

¹¹

,

Marta Tykwińska

¹²

,

Ewa Czarnobilska

¹

,

Marcin Moniuszko

¹³

,

Krystyna Obtułowicz

¹

Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
Department of Internal Diseases, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine – National Research Institute, Warsaw, Poland
1st Clinical Department of Pediatrics, Allergology and Cardiology, Wroclaw Medical University, Wroclaw, Poland
ALERGODERM, Specialist Medical Practice, Bydgoszcz, Poland
Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland
Greater Poland Center of Pulmonology and Thoracic Surgery, Poznan, Poland
Department of Immunology and Allergy, Central Teaching Hospital of the Medical University of Lodz, Poland
Department of Pulmonology and Allergology, Faculty of Medicine, Medical University of Gdansk, Poland
1st Department of Lung Diseases and Tuberculosis, Medical University of Bialystok, Poland
Independent Public Clinical Hospital No. 2, Pomeranian Medical University, Szczecin, Poland
Department of Allergology and Lung Diseases, Cardinal Wyszyński Provincial Specialist Hospital SPZOZ, Lublin, Poland
Department of Allergology, Clinical Immunology and Internal Medicine, University Hospital No. 2, Bydgoszcz, Poland
Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland

Alergologia Polska – Polish Journal of Allergology 2025; 12, 3: 153–162

DOI: https://doi.org/10.5114/pja.2025.153716

Online publish date: 2025/08/21

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AMA

Porębski G, Kucharczyk A, Matuszewski T, et al. Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis. Alergologia Polska - Polish Journal of Allergology. 2025;12(3):153-162. doi:10.5114/pja.2025.153716.

APA

Porębski, G., Kucharczyk, A., Matuszewski, T., Danielewicz, H., Gocki, J.,  & Grzela, K. et al. (2025). Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis. Alergologia Polska - Polish Journal of Allergology, 12(3), 153-162. https://doi.org/10.5114/pja.2025.153716

Chicago

Porębski, Grzegorz, Aleksandra Kucharczyk, Tomasz Matuszewski, Hanna Danielewicz, Jacek Gocki, Katarzyna Grzela,  and Aldona Juchacz et al. 2025. "Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis". Alergologia Polska - Polish Journal of Allergology 12 (3): 153-162. doi:10.5114/pja.2025.153716.

Harvard

Porębski, G., Kucharczyk, A., Matuszewski, T., Danielewicz, H., Gocki, J., Grzela, K., Juchacz, A., Kurowski, M., Kuziemski, K., Łukaszyk, M., Sokołowska, M., Stobiecki, M., Trębas-Pietraś, E., Tykwińska, M., Czarnobilska, E., Moniuszko, M.,  and Obtułowicz, K. (2025). Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis. Alergologia Polska - Polish Journal of Allergology, 12(3), pp.153-162. https://doi.org/10.5114/pja.2025.153716

MLA

Porębski, Grzegorz et al. "Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis." Alergologia Polska - Polish Journal of Allergology, vol. 12, no. 3, 2025, pp. 153-162. doi:10.5114/pja.2025.153716.

Vancouver

Porębski G, Kucharczyk A, Matuszewski T, Danielewicz H, Gocki J, Grzela K et al. Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis. Alergologia Polska - Polish Journal of Allergology. 2025;12(3):153-162. doi:10.5114/pja.2025.153716.

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Hereditary angioedema due to a genetically determined deficiency of the C1 complement inhibitor (HAE-C1INH) is a rare disease that significantly impairs daily functioning and may be life-threatening in severe cases. Angioedema attacks are triggered by a transient, localized increase in bradykinin levels and may affect the skin as well as the mucous membranes lining the gastrointestinal and respiratory tracts. The first recommendations of the Polish expert group on HAE-C1INH were published in 2018. The dynamic advancement of medical knowledge and the emergence of new therapeutic options created a strong need to revise and update these recommendations. This updated position statement by the HAE Section of the Polish Society of Allergology is based on the latest medical evidence on HAE-C1INH and takes into account local healthcare conditions. Part I of the document addresses clinical presentation, pathophysiology, diagnostic criteria, differential diagnosis, and the classification of HAE-C1INH within the current angioedema classification system.

keywords:

Position statement of the hereditary angioedema Section of the Polish Society of Allergology on the management of hereditary angioedema due to C1 inhibitor deficiency: 2025 update. Part I: Classification, pathophysiology, clinical presentation, diagnosis, and differential diagnosis

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