Alergologia Polska - Polish Journal of Allergology

Abstract

3/2025 vol. 12
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
  1. Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
  2. Department of Internal Diseases, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine – National Research Institute, Warsaw, Poland
  3. 1st Clinical Department of Pediatrics, Allergology and Cardiology, Wroclaw Medical University, Wroclaw, Poland
  4. ALERGODERM, Specialist Medical Practice, Bydgoszcz, Poland
  5. Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland
  6. Greater Poland Center of Pulmonology and Thoracic Surgery, Poznan, Poland
  7. Department of Immunology and Allergy, Central Teaching Hospital of the Medical University of Lodz, Poland
  8. Department of Pulmonology and Allergology, Faculty of Medicine, Medical University of Gdansk, Poland
  9. 1st Department of Lung Diseases and Tuberculosis, Medical University of Bialystok, Poland
  10. Independent Public Clinical Hospital No. 2, Pomeranian Medical University, Szczecin, Poland
  11. Department of Allergology and Lung Diseases, Cardinal Wyszyński Provincial Specialist Hospital SPZOZ, Lublin, Poland
  12. Department of Allergology, Clinical Immunology and Internal Medicine, University Hospital No. 2, Bydgoszcz, Poland
  13. 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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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

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

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