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Polyclonal autoantibodies against C1 inhibitor in a case of acquired angioedema
Isabel M Ponce1, Teresa Caballero, Marta Reche
1Immunology Unit, Hospital Universitario La Paz, Madrid, Spain.
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.