Russian Journal of AllergyRussian Journal of Allergy1810-88302686-682XFarmarus Print Media144710.36691/RJA1447Research ArticleHereditary angioedema. Clinical guidelines. (D84.1)BliznetzElena A.<p>Cand. Sci. (Med.), Senior Research Associate</p>bliznetzelena@mail.ruhttps://orcid.org/0000-0002-5339-5566ViktorovaEkaterina A.<p>MD, Cand. Sci.(Med.)</p>vikat.10@mail.ruhttps://orcid.org/0000-0002-2427-1417VishnevaElena A.<p>Dr. Sci. (Med.)</p>vishneva.e@yandex.ruhttps://orcid.org/0000-0001-7398-0562DzhobavaEliso M.<p>Dr. Sci. (Med.), Professor</p>super.lis9@yandex.ruhttps://orcid.org/0000-0003-3841-4762Il'inaNatalya I.<p>MD, Dr. Sci. (Med.), Professor</p>instimmun@yandex.ruhttps://orcid.org/0000-0002-3556-969XKuzmenkoNatalia B.<p>Cand. Sci.(Med.)</p>plunge@list.ruhttps://orcid.org/0000-0002-1669-8621LatyshevaElena A.<p>MD, Dr. Sci. (Med.), Senior Research Associate</p>ealat@mail.ruhttps://orcid.org/0000-0002-1606-205XLatyshevaTatiana V.<p>MD, Dr. Sci. (Med.), Professor</p>tvlat@mail.ruhttps://orcid.org/0000-0003-1508-0640MantoIrina A.<p>MD, Research Associate</p>irina.manto@yandex.ruhttps://orcid.org/0000-0001-6432-394XNamazova-BaranovaLeyla S.<p>Dr. Sci. (Med.), Professor</p>leyla.s.namazova@gmail.comhttps://orcid.org/0000-0002-2209-7531NenashevaNatalia M.<p> Professor, Doctor of Medical Sciences</p>1444031@gmail.comhttps://orcid.org/0000-0002-3162-2510PampuraAlexander N.<p>MD, Dr. Sci. (Med.), Professor. Veltischev Research and Clinical Institute for Pediatrics</p>apampura1@mail.ruhttps://orcid.org/0000-0001-5039-8473PolyakovAleksander V.<p>Dr. Sci. (Bio), Professor</p>apol@dnalab.ruhttps://orcid.org/0000-0002-0105-1833SelimzianovaLilia R.<p>Cand. Sci.(Med.), Senior Research Associate</p>lilysir@mail.ruhttps://orcid.org/0000-0002-3678-7939ShcherbinaAnna Yu.<p>professor, head of Immunology Department Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), PhD</p>shcher26@hotmail.comhttps://orcid.org/0000-0002-3113-4939Federal State Budgetary Institution Research Centre for Medical GeneticsDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and ImmunologyResearch Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences»Pirogov Russian National Research Medical UniversityLLC group of clinics Crede ExpertRussian Assotiation of Allergology and Clinical ImmunologyNational Research Center – Institute of Immunology Federal Medical-Biological Agency of RussiaMoscow State University of Medicine and Dentistry named after A.I. EvdokimovRussian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian FederationPirogov Russian National Research MedicalCentral Clinical Hospital of the Russian Academy of SciencesThe First Sechenov Moscow State Medical University (Sechenov University)Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology26062021182771141404202119042021Copyright © 2021, Pharmarus Print Media2021<p>Hereditary angioedema is a rare, potentially life-threatening genetic disease that manifests with skin and mucous/submucosal swelling that occurs under the influence of bradykinin. The clinical guidelines for hereditary angioedema (code for the International Statistical Classification of Diseases and Related Health Problems D84.1) were developed in December 2020 by the Russian Association of Allergology and Clinical Immunology, Association of Medical Geneticists, National Association of Experts in the Field of Primary Immunodeficiencies, Union of Pediatricians of Russia. In December 2020, the project was approved at a meeting of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (December 10, 2020 No. 743/12).</p>
<p>The clinical guidelines are devoted to optimizing the clinical care of patients with hereditary angioedema. The topic is relevant owing to the under-diagnosis of hereditary angioedema and insufficient awareness of doctors of various specialties about this problem, as well as the difficulties in managing this category of patients. The clinical guidelines contain information about the epidemiology, etiology, classification, pathogenesis, and common clinical manifestations of hereditary angioedema. Thus, the paper described actual diagnostic approaches and differential diagnosis of hereditary angioedema and established its diagnostic criteria. Medical care algorithms to patients with hereditary angioedema at the prehospital and hospital stages are also described. The principles of pharmacotherapy, including long-term prophylaxis, short-term prophylaxis, and acute therapy, for hereditary angioedema are also presented. Treatment regimens are stratified according to patients age and gender. Non-drug measures for the prevention hereditary angioedema attacks are considered. Sections were also added in the edition of the new clinical guidelines of hereditary angioedema. Clinical guidelines for hereditary angioedema are intended for practicing doctors of all specialties, students and teachers of medical universities, residents, graduate students of medical universities.</p>angioedemahereditary angioedemaC1-inhibitorbradykininclinical guidelinesангиоотёкнаследственный ангиоотёкС1-ингибиторбрадикининклиничекие рекомендации[Maurer M. Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema – The 2017 revision and update. Allergy. 2018;73(8):1575–1596. doi: 10.1111/all.13384][Caballero T, Baeza ML, Cabañas R, et al. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. 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