Is angioedema mast cell mediators-induced or bradykinin-induced? Challenges of differential diagnosis shown by a clinical case series

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access


Angioedema occurs due to a local increase in vascular permeability induced by mediators: bradykinin or mast cell mediators. The main challenge of the differential diagnosis of angioedema lies in the fact that different pathophysiological processes may lead to a very similar clinical picture. At the same time, verification of the type of angioedema is crucially important for the proper therapy selection. In 2020, a school for doctors "Chronic urticaria: scientific and medical accomplishments and practical aspects of patient management" was held on the base of the National Research Center Institute of Immunology of the FMBA of Russia as part of the work of the reference center that provides expert assistance to patients with urticaria (GA²LEN UCARE). A clinical cases series of patients related to the same family with Hereditary Angioedema with a mutation in the PLG gene was presented at the event in order to demonstrate the challenges of differential diagnosis between different types of angioedema. This article focused on the description of this series.

Full Text

Restricted Access

About the authors

Irina A. Manto

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

ORCID iD: 0000-0001-6432-394X
SPIN-code: 7944-5159
Russian Federation, 24, Kashirskoye shosse, Moscow, 115522

Elena A. Latysheva

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

ORCID iD: 0000-0002-1606-205X
SPIN-code: 2063-7973

MD, Dr. Sci. (Med.)

Russian Federation, 24, Kashirskoye shosse, Moscow, 115522

Daria O. Timoshenko

Pirogov Russian National Research Medical University (Pirogov Medical University), 1, Ostrovityanova, Moscow

Author for correspondence.
ORCID iD: 0000-0002-7585-1390
SPIN-code: 2714-0906
Russian Federation, 117997, 1, Ostrovityanova, Moscow, Russia

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

ORCID iD: 0000-0003-1508-0640
SPIN-code: 8929-7644

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

Russian Federation, 24, Kashirskoye shosse, Moscow, 115522; 20, building 1, Delegatskaya str., Moscow, 127473


  1. Depetri F, Tedeschi A, Cugno M. Angioedema and emergency medicine: From pathophysiology to diagnosis and treatment. Eur. J. Intern. Med. Elsevier. 2019;59:8–13. doi: 10.1016/j.ejim.2018.09.004.
  2. Maurer M, Magerl M. Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell–Mediated Angioedema. Clin. Rev. Allergy Immunol. Springer US. 2021. doi: 10.1007/s12016-021-08841-w.
  3. Obtułowicz K. Bradykinin-mediated angioedema. Pol Arch Med Wewn. 2016;126(1-2):76-85. doi: 10.20452/pamw.3273.
  4. Cicardi M, Suffritti C, Perego F, Caccia S. Novelties in the Diagnosis and Treatment of Angioedema. J Investig Allergol Clin Immunol. 2016;26(4):212-221. doi: 10.18176/jiaci.0087.
  5. Zuraw BL, Christiansen SC. HAE Pathophysiology and Underlying Mechanisms. Clin. Rev. Allergy Immunol. 2016;51(2):216–229. doi: 10.1007/s12016-016-8561-8.
  6. Maurer M, Magerl M, Ansotegui I et al. The international WAO/EAACI guideline for the management of hereditary angioedema - The 2017 revision and update. World Allergy Organ. J. 2018;11(1):1–20. doi: 10.1186/s40413-017-0180-1.
  7. Hereditary angioedema. Clinical guidelines (D84.1). 2020. (in Russian).
  8. Bork K, Machnig T, Wulff K et al. Clinical features of genetically characterized types of hereditary angioedema with normal C1 inhibitor: a systematic review of qualitative evidence. Orphanet J. Rare Dis. 2020;15(1):1–14. doi: 10.1186/s13023-020-01570-x.
  9. Bork K, Wulff K, Steinmüller-Magin L et al. Hereditary angioedema with a mutation in the plasminogen gene. Allergy Eur. J. Allergy Clin. Immunol. 2018;73(2):442–450. doi: 10.1111/all.13270.
  10. Banday AZ, Kaur A, Jindal AK et al. An update on the genetics and pathogenesis of hereditary angioedema. Genes Dis. Elsevier Ltd. 2020;7(1):75–83. doi: 10.1016/j.gendis.2019.07.002.
  11. Bodian DL, Vilboux T, Hauser NS. Genotype-first analysis of a generally healthy population cohort supports genetic testing for diagnosis of hereditary angioedema of unknown cause. Allergy, Asthma Clin. Immunol. BioMed Central. 2019;15(1):1–4. doi: 10.1186/s13223-019-0346-1.

Supplementary files

There are no supplementary files to display.



Abstract: 129

Article Metrics

Metrics Loading ...



Copyright (c) Russian Journal of Allergy

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies