C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency [PDF]
Orphanet Journal of Rare Diseases, 2023 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
Zsofia Polai +6 more
doaj +4 more sources
Editorial: C1 inhibitor deficiency and angioedema [PDF]
Frontiers in Allergy, 2022 Henriette Farkas +2 more
doaj +4 more sources
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate [PDF]
Orphanet Journal of Rare Diseases, 2019 Background Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal edema. It is associated with malignant B-cell lymphoma and other disorders.
Konrad Bork +2 more
doaj +4 more sources
C1 inhibitor deficiency: consensus document [PDF]
Clinical and Experimental Immunology, 2005 SummaryWe present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein.
M Abinun, R J Lock
exaly +3 more sources
Daratumumab-based treatment of monoclonal gammopathy–associated angioedema due to acquired C1-inhibitor deficiency [PDF]
Journal of Allergy and Clinical Immunology: GlobalDaratumumab-based treatment could control severe, treatment-refractory, life-threatening angioedema due to acquired C1-inhibitor deficiency associated with monoclonal gammopathy.
Remy S. Petersen, MD +4 more
doaj +2 more sources
Searching for Genetic Biomarkers for Hereditary Angioedema Due to C1-Inhibitor Deficiency (C1-INH-HAE) [PDF]
Frontiers in Allergy, 2022 Existing evidence indicates that modifier genes could change the phenotypic outcome of the causal SERPING1 variant and thus explain the expression variability of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE).
Faidra Parsopoulou +18 more
doaj +2 more sources
Lymphoproliferative disease and acquired C1 inhibitor deficiency
Haematologica, 2007 Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal ...
Roberto Castelli +4 more
doaj +7 more sources
13th C1-inhibitor deficiency and angioedema workshop—2023 [PDF]
Allergy, Asthma & Clinical Immunology, 2023 doaj +2 more sources
Secreted Phospholipases A2 in Hereditary Angioedema With C1-Inhibitor Deficiency
Frontiers in Immunology, 2018 BackgroundHereditary angioedema (HAE) caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) is a disabling, potentially fatal condition characterized by recurrent episodes of swelling. We have recently found that
Stefania Loffredo +16 more
doaj +3 more sources
Abstracts of the 14th C1-inhibitor Deficiency and Angioedema Workshop [PDF]
Allergy, Asthma & Clinical Immunologydoaj +2 more sources