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231225s2021 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2021.108819
|2 doi
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|a pubmed24n1096.xml
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|a (NLM)34358691
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|a (PII)S1521-6616(21)00156-X
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|a DE-627
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|a eng
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| 100 |
1 |
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|a Shi, Yiyun
|e verfasserin
|4 aut
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| 245 |
1 |
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|a Where we are with acquired angioedema due to C1 inhibitor deficiency
|b A systematic literature review
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|c 2021
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| 336 |
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
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|2 rdamedia
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|a ƒa Online-Ressource
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|a Date Completed 13.09.2021
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|a Date Revised 13.09.2021
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2021 Elsevier Inc. All rights reserved.
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|a Acquired angioedema due to C1 inhibitor deficiency (C1-INH-AAE) is a rare disease characterized by adult-onset recurrent non-urticarial angioedema with low levels of C1-INH. It is associated with lymphoproliferative disorders, and treatments are off-label with variable success. We conducted a systematic literature review to include patients with C1-INH-AAE identified in PubMed and Embase databases between January 2006 and February 2021. Clinical features of these patients were summarized, and factors associated with disease remission were explored. A total of 121 patients were included in the current study with a median age at diagnosis of 64 years and 45.5% being male. An associated disease was recorded in 94 patients (77.7%), and lymphoproliferative disorder was the most reported (59/94, 62.8%). Anti-C1-INH autoantibodies were present in 45 of 71 patients (63.4%). Factors impacting disease remissions included age (odds ratio [OR] 0.951, 95% confidence interval [CI] 0.909-0.994, p = 0.027), male (OR 0.327, 95% CI 0.124-0.866, p = 0.025), presence of monoclonal gammopathy (OR 0.133, 95% CI 0.041-0.429, p = 0.001), requirement of specific on-demand treatment (OR 0.216, 95% CI 0.066-0.709, p = 0.012) and rituximab use (OR 2.865, 95% CI 1.038-7.911, p = 0.042). A total of nine patients (7.4%) died at last follow up and most were unrelated to C1-INH-AAE. Our results imply that C1-INH-AAE is primarily associated with underlying B or plasma cell abnormalities, and clone-directed therapies could be promising options for its long-term management
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|a Journal Article
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|a Systematic Review
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|a Acquired angioedema
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|a C1 inhibitor
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| 650 |
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|a Lymphoproliferative disorder
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| 650 |
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|a Rituximab
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| 650 |
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|a Autoantibodies
|2 NLM
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| 650 |
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|a Complement C1
|2 NLM
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| 650 |
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|a Complement C1 Inhibitor Protein
|2 NLM
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| 650 |
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|a SERPING1 protein, human
|2 NLM
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| 700 |
1 |
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|a Wang, Chen
|e verfasserin
|4 aut
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| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 230(2021) vom: 06. Sept., Seite 108819
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|g pages:108819
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|u http://dx.doi.org/10.1016/j.clim.2021.108819
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