Background: Angioedema attributable to acquired C1 inhibitor (C1-INH) deficiency is a rare disease related to lymphoproliferative disorders or autoantibodies to Cl inhibitor. We describe a patient with angioedema and autoantibodies to C1 inhibitor.
Objective: To study the characteristics of autoantibodies to C1-INH in a patient with acquired angioedema.
Methods: Autoantibodies to Cl-INH were measured by enzyme-linked immunoadsorbent assay. Immunoglobulin (Ig)G autoantibody was purified by affinity chromatography on a protein G agarose column. We developed an enzyme-linked immunoadsorbent assay to determine whether the autoantibodies were directed against the C1-INH active center.
Results: IgM and mainly C1-INH IgG autoantibodies were detected; both had kappa and lambda chains. No monoclonal component was detected. The autoantibodies were directed against the Cl-INH active center. After various treatment strategies were attempted, an effective clinical response was attained with antifibrinolytic therapy.
Conclusion: A case of acquired angioedema because of C1-INH deficiency was found to be attributable to the presence of polyclonal autoantibodies to C1-INH.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/S1081-1206(10)61896-0 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Hypomorphic RAG2 Deficiency Promotes Selection of Self-Reactive B Cells.
J Clin Immunol
January 2025
Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA, USA.
Reduced function or hypomorphic variants in recombination-activating genes (RAG) 1 or 2 result in a broad clinical phenotype including common variable immunodeficiency (CVID) and even adult-onset disease. Milder RAG variants are less characterized. Here we describe the longitudinal course of a milder combined RAG deficiency in 3 of 7 siblings sharing the same RAG2 mutations over a 50-year study.
View Article and Find Full Text PDFNeuro-immunobiology and treatment assessment in a mouse model of anti-NMDAR encephalitis.
Brain
December 2024
Neuroimmunology Program, Fundació Clínic per la Recerca Biomèdica - Institut d'Investigacions Biomèdiques August Pi i Sunyer (FCRB-IDIBAPS), Barcelona 08036, Spain.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disorder mediated by autoantibodies against the GluN1 subunit of NMDAR. It occurs with severe neuropsychiatric symptoms that often improve with immunotherapy. Clinical studies and animal models based on patients' antibody transfer or NMDAR immunization suggest that the autoantibodies play a major pathogenic role.
View Article and Find Full Text PDFAnti-Centromere Protein A Antibody Disrupts the Competence of Mouse Oocytes Matured In Vitro.
Am J Reprod Immunol
December 2024
Department of Obstetrics and Gynecology, Reproductive Medicine, Graduate School of Medicine, Chiba University, Chuo-ku, Chiba, Japan.
Introduction: Anticentromere autoantibodies are associated with refractory IVF/ET failure, but causality is unclear. Experimental models are needed.
Methods: Immature oocytes collected from 23-day-old mice were matured in vitro for 18 h in a culture medium containing an anti-human centromere protein A (CENP-A) polyclonal antibody, and those oocytes' maturity and chromosome/spindle structure were assessed.
Cross-reactivity of antigenic binding sites of antiphosphatidylserine/prothrombin antibodies in patients with pregnancy loss and epidermal growth factor.
J Reprod Immunol
November 2024
Laboratory for Recurrent Pregnancy Loss, Sugi Women's Clinic, 2-12-1-7 F Shinyokohama, Kohoku, Yokohama, Kanagawa 222-0033, Japan.
Background: Epidermal growth factor (EGF) protein family is essential for implantation and maintenance of normal pregnancy. Results of our previous study on the high incidence of autoantibodies to EGF in patients with pregnancy loss indicated a strong association between EGF autoantibodies and antiphosphatidylserine/prothrombin antibodies (aPS/PT), which are observed in the plasma of patients with thrombosis and adverse pregnancy outcomes.
Objectives: To investigate the association between EGF autoantibodies and aPS/PT in patients with pregnancy loss.
IFN-associated B cell hyperactivity is highly enriched in SLE patients harboring Sm/RNP antibodies.
bioRxiv
November 2024
Department of rheumatology, Leiden University Medical Center, Leiden, The Netherlands.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by an array of autoantibodies, in particular anti-nuclear antibodies (ANA). The disease is also hallmarked by an expansion of plasmablasts (PB) in peripheral blood. How these relate to autoantibody production is not clear.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!