Ponce Isabel M, Caballero Teresa, Reche Marta, Piteiro Ana B, López-Serrano M Concepción, Fontán Gumersindo, López-Trascasa Margarita
Immunology Unit, Hospital Universitario La Paz, Madrid, Spain.
Ann Allergy Asthma Immunol. 2002 Jun;88(6):632-7. doi: 10.1016/S1081-1206(10)61896-0.
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.
To study the characteristics of autoantibodies to C1-INH in a patient with acquired angioedema.
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.
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.
A case of acquired angioedema because of C1-INH deficiency was found to be attributable to the presence of polyclonal autoantibodies to C1-INH.
获得性C1抑制物（C1-INH）缺乏所致血管性水肿是一种与淋巴增殖性疾病或C1抑制物自身抗体相关的罕见疾病。我们描述了一名患有血管性水肿且存在C1抑制物自身抗体的患者。
研究一名获得性血管性水肿患者体内C1-INH自身抗体的特征。
采用酶联免疫吸附测定法检测C1-INH自身抗体。通过蛋白G琼脂糖柱亲和层析纯化免疫球蛋白（Ig）G自身抗体。我们开发了一种酶联免疫吸附测定法来确定自身抗体是否针对C1-INH活性中心。
检测到IgM以及主要为C1-INH IgG自身抗体；两者均有κ链和λ链。未检测到单克隆成分。自身抗体针对C1-INH活性中心。尝试了各种治疗策略后，抗纤溶治疗取得了有效的临床反应。
发现一例因C1-INH缺乏导致的获得性血管性水肿病例是由于存在针对C1-INH的多克隆自身抗体。
