Hereditary angioedema. Clinical guidelines. (D84.1)

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  • Authors: Bliznetz E.A.1, Viktorova E.A.2, Vishneva E.A.3,4, Dzhobava E.M.5, Il'ina N.I.6,7, Kuzmenko N.B.2, Latysheva E.A.7, Latysheva T.V.7,8, Manto I.A.7, Namazova-Baranova L.S.3,4, Nenasheva N.M.9, Pampura A.N.10, Polyakov A.V.1, Selimzianova L.R.11,4,12, Shcherbina A.Y.13
  • Affiliations:
    1. Federal State Budgetary Institution Research Centre for Medical Genetics
    2. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
    3. Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences»
    4. Pirogov Russian National Research Medical University
    5. LLC group of clinics Crede Expert
    6. Russian Assotiation of Allergology and Clinical Immunology
    7. National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia
    8. Moscow State University of Medicine and Dentistry named after A.I. Evdokimov
    9. Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation
    10. Pirogov Russian National Research Medical
    11. Central Clinical Hospital of the Russian Academy of Sciences
    12. The First Sechenov Moscow State Medical University (Sechenov University)
    13. Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
  • Issue: Vol 18, No 2 (2021)
  • Pages: 77-114
  • Section: Help to the practitioner
  • URL: https://journal-vniispk.ru/raj/article/view/121766
  • DOI: https://doi.org/10.36691/RJA1447
  • ID: 121766

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Full Text

Abstract

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).

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.

About the authors

Elena A. Bliznetz

Federal State Budgetary Institution Research Centre for Medical Genetics

Email: bliznetzelena@mail.ru
ORCID iD: 0000-0002-5339-5566
SPIN-code: 8451-3075

Cand. Sci. (Med.), Senior Research Associate

Russian Federation, Moscow

Ekaterina A. Viktorova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: vikat.10@mail.ru
ORCID iD: 0000-0002-2427-1417

MD, Cand. Sci.(Med.)

Russian Federation, Moscow

Elena A. Vishneva

Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences»; Pirogov Russian National Research Medical University

Email: vishneva.e@yandex.ru
ORCID iD: 0000-0001-7398-0562
SPIN-code: 1109-2810

Dr. Sci. (Med.)

Russian Federation, Moscow; Moscow

Eliso M. Dzhobava

LLC group of clinics Crede Expert

Email: super.lis9@yandex.ru
ORCID iD: 0000-0003-3841-4762
SPIN-code: 8866-4534

Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Natalya I. Il'ina

Russian Assotiation of Allergology and Clinical Immunology; National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia

Email: instimmun@yandex.ru
ORCID iD: 0000-0002-3556-969X
SPIN-code: 6715-5650

MD, Dr. Sci. (Med.), Professor

Russian Federation, 24 Kashirskoe shosse, 115522, Moscow; Moscow

Natalia B. Kuzmenko

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: plunge@list.ru
ORCID iD: 0000-0002-1669-8621

Cand. Sci.(Med.)

Russian Federation, Moscow

Elena A. Latysheva

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia; National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia

Email: ealat@mail.ru
ORCID iD: 0000-0002-1606-205X
SPIN-code: 2063-7973

MD, Dr. Sci. (Med.), Senior Research Associate

Russian Federation, Moscow; 24, Kashirskoye shosse, Moscow, 115522

Tatiana V. Latysheva

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia; Moscow State University of Medicine and Dentistry named after A.I. Evdokimov

Email: tvlat@mail.ru
ORCID iD: 0000-0003-1508-0640
SPIN-code: 8929-7644

MD, Dr. Sci. (Med.), Professor

Russian Federation, 24 Kashirskoe shosse, Moscow,115522; Moscow

Irina A. Manto

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia

Author for correspondence.
Email: irina.manto@yandex.ru
ORCID iD: 0000-0001-6432-394X
SPIN-code: 7944-5159

MD, Research Associate

Russian Federation, 24, Kashirskoye shosse, Moscow, 115522

Leyla S. Namazova-Baranova

Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences»; Pirogov Russian National Research Medical University

Email: leyla.s.namazova@gmail.com
ORCID iD: 0000-0002-2209-7531
SPIN-code: 1312-2147

Dr. Sci. (Med.), Professor

Russian Federation, Moscow; Moscow

Natalia M. Nenasheva

Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation

Email: 1444031@gmail.com
ORCID iD: 0000-0002-3162-2510
SPIN-code: 3363-6170

 Professor, Doctor of Medical Sciences

Russian Federation, 2/1 bld 1, Barrikadnaya ul., Moscow, 125993

Alexander N. Pampura

Pirogov Russian National Research Medical

Email: apampura1@mail.ru
ORCID iD: 0000-0001-5039-8473
SPIN-code: 9722-7961

MD, Dr. Sci. (Med.), Professor. Veltischev Research and Clinical Institute for Pediatrics

Russian Federation, Moscow

Aleksander V. Polyakov

Federal State Budgetary Institution Research Centre for Medical Genetics

Email: apol@dnalab.ru
ORCID iD: 0000-0002-0105-1833
SPIN-code: 6453-3097

Dr. Sci. (Bio), Professor

Russian Federation, Moscow

Lilia R. Selimzianova

Central Clinical Hospital of the Russian Academy of Sciences; Pirogov Russian National Research Medical University; The First Sechenov Moscow State Medical University (Sechenov University)

Email: lilysir@mail.ru
ORCID iD: 0000-0002-3678-7939
SPIN-code: 5508-1689

Cand. Sci.(Med.), Senior Research Associate

Russian Federation, Moscow; Moscow; Moscow

Anna Yu. Shcherbina

Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology

Email: shcher26@hotmail.com
ORCID iD: 0000-0002-3113-4939
SPIN-code: 6759-0031

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

Russian Federation, Moscow

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2. Fig. 1.The pathogenesis of hereditary angioedema with C1-inhibitor defi ciency (types I and II).

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