Long-term prophylaxis therapy in patients with hereditary angioedema in Russia: resolution of the expert Council

Olga S. Bodnya; Бодня Ольга Сергеевна; Darya V. Demina; Демина Дарья Владимировна; Natalia B. Kuzmenko; Кузьменко Наталья Борисовна; Elena A. Latysheva; Латышева Елена Александровна; Tatiana V. Latysheva; Латышева Татьяна Васильевна; Anzhelika M. Milichkina; Миличкина Анжелика Марсовна; Alexander N. Pampura; Пампура Александр Николаевич; Liudmila P. Sizyakina; Сизякина Людмила Петровна; Areg A. Totolian; Тотолян Арег Артемович; Olga P. Uhanova; Уханова Ольга Петровна; Natalia V. Fedotova; Федотова Наталья Викторовна; Daria S. Fomina; Фомина Дарья Сергеевна

doi:10.36691/RJA1485

Long-term prophylaxis therapy in patients with hereditary angioedema in Russia: resolution of the expert Council

Authors: Bodnya O.S.¹, Demina D.V.², Kuzmenko N.B.³, Latysheva E.A.⁴^,5, Latysheva T.V.⁴^,6, Milichkina A.M.⁷, Pampura A.N.⁸, Sizyakina L.P.⁹, Totolian A.A.⁷, Uhanova O.P.¹⁰, Fedotova N.V.¹¹, Fomina D.S.¹²^,13
Affiliations:
1. Russian Medical Academy of Continuous Professional Education
2. Federal State Budgetary Scientific Institution Research Institute of Fundamental and Clinical Immunology
3. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
4. National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia
5. Pirogov Russian National Research Medical University
6. Moscow State University of Medicine and Dentistry named after A.I. Evdokimov
7. Saint-Petersburg Pasteur Institute
8. Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
9. Rostov State Medical University
10. Stavropol State Medical University
11. Scientific Research Institute – Ochapovsky Regional Clinic Hospital of Krasnodar Region
12. Сlinical State Hospital №52
13. The First Sechenov Moscow State Medical University (Sechenov University)
Issue: Vol 18, No 3 (2021)
Pages: 126-130
Section: Letters to the editor
URL: https://journal-vniispk.ru/raj/article/view/121728
DOI: https://doi.org/10.36691/RJA1485
ID: 121728

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Abstract

Hereditary angioedema is a rare (orphan) disease associated with the development of angioedema of various localization caused by the action of bradykinin. The main symptoms of the disease are peripheral angioedema, abdominal attacks (accompanied by severe pain syndrome), edema of the upper respiratory tract, which may lead to asphyxia and death of the patient. Peripheral edema disrupts social and professional activity, the occurrence of facial edema complicates social adaptation, the unpredictability and potential threat to life of edema in the larynx, as well as the lack of effect from standard (systemic glucocorticoid and antihistamines) therapy regimens lead to a low quality of life of patients, a large number of days of disability, hospitalizations, in some cases, premature death of patients.

According to international studies, “on-demand” therapy does not reduce the burden of the disease, and therefore international guidelines emphasize the importance of timely and individualized selection of long-term prevention for patients with hereditary angioedema. Taking into account the appearance of a new drug for long-term prevention in Russia, an Expert Council was held to assess the burden of the disease in Russian patients, determine the proportion of patients who need long-term prevention, criteria for choosing a medication for prophylaxis and the place of Lanadelumab in it.

Keywords

hereditary angioedema, long-term prevention, criteria for prescribing long-term prevention, lanadelumab, clinical guidelines

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About the authors

Olga S. Bodnya

Russian Medical Academy of Continuous Professional Education

Email: os.bodnya@yandex.ru
ORCID iD: 0000-0001-5009-8251
SPIN-code: 8477-0538

MD, Cand. Sci. (Med.)

Russian Federation, Moscow

Darya V. Demina

Federal State Budgetary Scientific Institution Research Institute of Fundamental and Clinical Immunology

Email: immunology@mail.ru
ORCID iD: 0000-0002-0342-5368

MD, Cand. Sci. (Med.)

Russian Federation, Novosibirsk

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
SPIN-code: 6281-2729

MD, Dr. Sci. (Med)

Russian Federation, Moscow

Elena A. Latysheva

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

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

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

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

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, Kashirskoye shosse, Moscow, 115522; Moscow