Archives of Hellenic Medicine

Last update:

22-Apr-2024

Arch Hellen Med, 41(3), May-June 2024, 404-417

SPECIAL ARTICLE

Consensus recommendations on the diagnosis and treatment of hereditary angioedema
Hellenic Society of Angioedema*
*The group of experts involved equally in the formulation of the recommendations

A.E. Germenis (co -ordinator),¹ E. Kompoti,² G.N. Konstantinou,³ M. Makris,⁴ E. Manousakis,⁵ N. Mikos,² J. Paraskevopoulos,⁶ M. Speletas,¹ E. Stefanaki,⁷ E. Farmaki,⁸ F. Psarros⁹
¹Department of Immunology & Histocompatibility, School of Medicine, University of Thessaly, Larissa,
²Department of Allergology-Clinical Immunology, "Laikon" General Hospital of Athens, Athens,
³Department of Allergy and Clinical Immunology, 424 General Military Training Hospital, Thessaloniki,
⁴"D. Kalogeromitros" Allergy Unit, Second Department of Dermatology and Venereology, National and Kapodistrian University of Athens, "Attikon" University Hospital, Athens,
⁵Department of Allergy, Second Pediatric Clinic, National and Kapodistrian University of Athens, Athens,
⁶Allergy Outpatient Clinic, 401 General Military Hospital of Athens, Athens,
⁷Allergologist, Ioannina,
⁸First Department of Pediatrics, "Ippokration" General Hospital, Aristotle University of Thessaloniki, Thessaloniki,
⁹Department of Allergology, Naval Hospital of Athens, Athens, Greece

Hereditary angioedema is a rare disease manifesting with recurrent attacks of disabling and potentially life-threatening angioedema, for which early diagnosis and effective therapy are critical. Recent scientific progress and the development of novel therapeutic options has engendered multiple important changes in the diagnosis and management of the disease. In an attempt to encourage and facilitate the use of this progress for all patients, the Hellenic Society of Angioedema has undertaken the effort to develop consensus recommendations for the diagnosis, treatment, and management of hereditary angioedema in special patients' groups. To this aim, a panel of 11 experts was assembled and a modified Delphi method was used. After a comprehensive review of relevant literature from the last five years retrieved from Medline, the initial text of the recommendations was formulated and consensus was sought among the experts. The consensus included statements that were agreed by more than 80% of the experts. This article presents the 24 consensus recommendations that were finally formulated.

Key words: C1-esterase inhibitor deficiency, Hereditary angioedema, Long-term prophylaxis, Recommendations, Short-term prophylaxis, Unknown hereditary angioedema.