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@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.
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