C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency
Abstract Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim was to measure C1-INH/C1-INH-Ab comp...
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| Format: | Article |
| Language: | English |
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BMC
2023-02-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-023-02625-5 |
| _version_ | 1811171547024457728 |
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| author | Zsofia Polai Erika Kajdacsi Laszlo Cervenak Zsuzsanna Balla Szabolcs Benedek Lilian Varga Henriette Farkas |
| author_facet | Zsofia Polai Erika Kajdacsi Laszlo Cervenak Zsuzsanna Balla Szabolcs Benedek Lilian Varga Henriette Farkas |
| author_sort | Zsofia Polai |
| collection | DOAJ |
| description | Abstract Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim was to measure C1-INH/C1-INH-Ab complexes (CAC) and investigate their connection to C1-INH-Ab and the changes in their titer over time. Results 19 patients were diagnosed with C1-INH-AAE in the Hungarian Angioedema Center of Reference and Excellence; 79% of them had an underlying disease. Samples were examined with a newly developed in-house complex ELISA method. Patients with high C1-INH-Ab titer had a CAC titer which did not exceed the normal level and the ones with high CAC titer had a C1-INH-Ab titer which did not exceed the normal level. In case of those patients who had C1-INH-Ab and CAC of the same type of immunoglobulin, the increasing titer of C1-INH-Ab went together with the decreasing level of CAC and vice versa. CAC titer was already increased before the diagnosis of the underlying disease. Conclusions Free circulating and complex antibodies are in a dynamically changing equilibrium. CAC measurements can help to predict the development of an underlying disease. The efficiency of the treatment for underlying disease can be monitored by the decreasing CAC titers. Our results show that the CAC can be of important additional information besides the complement panel examination in case of C1-INH-AAE. Measurement of CAC is recommended to be done parallelly with C1-INH-Ab, so as to detect both free and bound antibodies. |
| first_indexed | 2024-04-10T17:15:50Z |
| format | Article |
| id | doaj.art-1255427f90a740baa050a589cd3387b3 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-10T17:15:50Z |
| publishDate | 2023-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-1255427f90a740baa050a589cd3387b32023-02-05T12:23:40ZengBMCOrphanet Journal of Rare Diseases1750-11722023-02-011811910.1186/s13023-023-02625-5C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiencyZsofia Polai0Erika Kajdacsi1Laszlo Cervenak2Zsuzsanna Balla3Szabolcs Benedek4Lilian Varga5Henriette Farkas6Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis UniversityDepartment of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis UniversityAbstract Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim was to measure C1-INH/C1-INH-Ab complexes (CAC) and investigate their connection to C1-INH-Ab and the changes in their titer over time. Results 19 patients were diagnosed with C1-INH-AAE in the Hungarian Angioedema Center of Reference and Excellence; 79% of them had an underlying disease. Samples were examined with a newly developed in-house complex ELISA method. Patients with high C1-INH-Ab titer had a CAC titer which did not exceed the normal level and the ones with high CAC titer had a C1-INH-Ab titer which did not exceed the normal level. In case of those patients who had C1-INH-Ab and CAC of the same type of immunoglobulin, the increasing titer of C1-INH-Ab went together with the decreasing level of CAC and vice versa. CAC titer was already increased before the diagnosis of the underlying disease. Conclusions Free circulating and complex antibodies are in a dynamically changing equilibrium. CAC measurements can help to predict the development of an underlying disease. The efficiency of the treatment for underlying disease can be monitored by the decreasing CAC titers. Our results show that the CAC can be of important additional information besides the complement panel examination in case of C1-INH-AAE. Measurement of CAC is recommended to be done parallelly with C1-INH-Ab, so as to detect both free and bound antibodies.https://doi.org/10.1186/s13023-023-02625-5Bradykinin-mediated angioedemaAcquired angioedema due to C1-inhibitor deficiencyC1-inhibitorAntibodies against C1-inhibitorComplement cascadeClassical pathway |
| spellingShingle | Zsofia Polai Erika Kajdacsi Laszlo Cervenak Zsuzsanna Balla Szabolcs Benedek Lilian Varga Henriette Farkas C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency Orphanet Journal of Rare Diseases Bradykinin-mediated angioedema Acquired angioedema due to C1-inhibitor deficiency C1-inhibitor Antibodies against C1-inhibitor Complement cascade Classical pathway |
| title | C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency |
| title_full | C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency |
| title_fullStr | C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency |
| title_full_unstemmed | C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency |
| title_short | C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency |
| title_sort | c1 inhibitor c1 inhibitor antibody complexes in acquired angioedema due to c1 inhibitor deficiency |
| topic | Bradykinin-mediated angioedema Acquired angioedema due to C1-inhibitor deficiency C1-inhibitor Antibodies against C1-inhibitor Complement cascade Classical pathway |
| url | https://doi.org/10.1186/s13023-023-02625-5 |
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