Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence,
Clinical Features, and Management: A Nationwide Retrospective
Study in the Czech Republic Patients

J 2021

Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients

SOBOTKOVA, Marta, Radana ZACHOVA, Roman HAKL, Pavel KUKLÍNEK, Pavlina KRALICKOVA et. al.

Basic information

Original name

Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients

Authors

SOBOTKOVA, Marta (203 Czech Republic, guarantor), Radana ZACHOVA (203 Czech Republic), Roman HAKL (203 Czech Republic, belonging to the institution), Pavel KUKLÍNEK (203 Czech Republic, belonging to the institution), Pavlina KRALICKOVA (203 Czech Republic), Irena KRCMOVA (203 Czech Republic), Jana HANZLIKOVA (203 Czech Republic), Martina VACHOVA (203 Czech Republic) and Jirina BARTUNKOVA (203 Czech Republic)

Edition

International archives of allergy and immunology, Basel, Karger, 2021, 1018-2438

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30102 Immunology

Country of publisher

Switzerland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 3.767

RIV identification code

RIV/00216224:14110/21:00121149

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1159/000512933

UT WoS

000609314400001

Keywords in English

Acquired angioedema; C1 inhibitor; Bradykinin; Lymphoma

Abstract

V originále

Introduction: Acquired angioedema with C1 inhibitor deficiency (AAE-C1-INH) is rare but a potentially life-threatening disease. There are no official prevalence data, nor approved therapies for this condition. Objective: In this study, we aimed to collect and analyze clinical data on patients with AAE-C1-INH in the Czech Republic. Methods: We have conducted a retrospective analysis of AAE-C1-INH patients from Czech referral centers for the treatment of hereditary angioedema with C1 inhibitor deficiency. The inclusion criteria involved recurrent episodes of angioedema with the first manifestation at or after the age of 40, negative family history of angioedema, and C1 inhibitor function 50% or less. Results: A total of 14 patients (7 males and 7 females) met the inclusion criteria for AAE-C1-INH. The median age of the symptom onset was 59.5 years, and the median diagnosis delay was 1 year. The most common clinical manifestation was facial edema (100%) and upper airway swelling (85.7%). All patients responded to the acute attack treatment with icatibant and plasma-derived or recombinant C1 inhibitor concentrate. Lymphoid malignancy was identified in 9 patients (64%), monoclonal gammopathy of uncertain significance in 3 (21%), and in 1 patient autoimmune disease (ulcerative colitis) was considered causative (7%). We were not able to identify any underlying disease only in 1 patient (7%). In 6 of 7 patients (86%) treated for lymphoma, either a reduction in the frequency of angioedema attacks or both angioedema symptoms' disappearance and complement parameter normalization was observed. Conclusions: The prevalence of AAE-C1-INH in the Czech Republic is about 1:760,000. This rare condition occurs in approximately 8% of the patients with angioedema with C1 inhibitor deficiency. AAE-C1-INH is strongly associated with lymphoproliferative disorders, and treating these conditions may improve the control of angioedema symptoms.

Citovat

SOBOTKOVA, Marta, Radana ZACHOVA, Roman HAKL, Pavel KUKLÍNEK, Pavlina KRALICKOVA, Irena KRCMOVA, Jana HANZLIKOVA, Martina VACHOVA and Jirina BARTUNKOVA. Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients. International archives of allergy and immunology. Basel: Karger, 2021, vol. 182, No 7, p. 642-649. ISSN 1018-2438. Available from: https://dx.doi.org/10.1159/000512933.

@article{1743549,
   author = {Sobotkova, Marta and Zachova, Radana and Hakl, Roman and Kuklínek, Pavel and Kralickova, Pavlina and Krcmova, Irena and Hanzlikova, Jana and Vachova, Martina and Bartunkova, Jirina},
   article_location = {Basel},
   article_number = {7},
   doi = {http://dx.doi.org/10.1159/000512933},
   keywords = {Acquired angioedema; C1 inhibitor; Bradykinin; Lymphoma},
   language = {eng},
   issn = {1018-2438},
   journal = {International archives of allergy and immunology},
   title = {Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients},
   url = {https://www.karger.com/Article/Pdf/512933},
   volume = {182},
   year = {2021}
}

TY  - JOUR
ID  - 1743549
AU  - Sobotkova, Marta - Zachova, Radana - Hakl, Roman - Kuklínek, Pavel - Kralickova, Pavlina - Krcmova, Irena - Hanzlikova, Jana - Vachova, Martina - Bartunkova, Jirina
PY  - 2021
TI  - Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients
JF  - International archives of allergy and immunology
VL  - 182
IS  - 7
SP  - 642-649
EP  - 642-649
PB  - Karger
SN  - 10182438
KW  - Acquired angioedema
KW  - C1 inhibitor
KW  - Bradykinin
KW  - Lymphoma
UR  - https://www.karger.com/Article/Pdf/512933
L2  - https://www.karger.com/Article/Pdf/512933
N2  - Introduction: Acquired angioedema with C1 inhibitor deficiency (AAE-C1-INH) is rare but a potentially life-threatening disease. There are no official prevalence data, nor approved therapies for this condition. Objective: In this study, we aimed to collect and analyze clinical data on patients with AAE-C1-INH in the Czech Republic. Methods: We have conducted a retrospective analysis of AAE-C1-INH patients from Czech referral centers for the treatment of hereditary angioedema with C1 inhibitor deficiency. The inclusion criteria involved recurrent episodes of angioedema with the first manifestation at or after the age of 40, negative family history of angioedema, and C1 inhibitor function 50% or less. Results: A total of 14 patients (7 males and 7 females) met the inclusion criteria for AAE-C1-INH. The median age of the symptom onset was 59.5 years, and the median diagnosis delay was 1 year. The most common clinical manifestation was facial edema (100%) and upper airway swelling (85.7%). All patients responded to the acute attack treatment with icatibant and plasma-derived or recombinant C1 inhibitor concentrate. Lymphoid malignancy was identified in 9 patients (64%), monoclonal gammopathy of uncertain significance in 3 (21%), and in 1 patient autoimmune disease (ulcerative colitis) was considered causative (7%). We were not able to identify any underlying disease only in 1 patient (7%). In 6 of 7 patients (86%) treated for lymphoma, either a reduction in the frequency of angioedema attacks or both angioedema symptoms' disappearance and complement parameter normalization was observed. Conclusions: The prevalence of AAE-C1-INH in the Czech Republic is about 1:760,000. This rare condition occurs in approximately 8% of the patients with angioedema with C1 inhibitor deficiency. AAE-C1-INH is strongly associated with lymphoproliferative disorders, and treating these conditions may improve the control of angioedema symptoms.
ER  -

SOBOTKOVA, Marta, Radana ZACHOVA, Roman HAKL, Pavel KUKLÍNEK, Pavlina KRALICKOVA, Irena KRCMOVA, Jana HANZLIKOVA, Martina VACHOVA and Jirina BARTUNKOVA. Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients. \textit{International archives of allergy and immunology}. Basel: Karger, 2021, vol.~182, No~7, p.~642-649. ISSN~1018-2438. Available from: https://dx.doi.org/10.1159/000512933.

Detailed Information on Publication Record