Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy

Shinji HIGA; Haruhiko HIRATA; Seigou MINAMI; Shouji HASHIMOTO; Masaki SUEMURA; Yukihiko SAEKI; Ichiro KAWASE; Toshio TANAKA

doi:10.2169/internalmedicine.41.398

Shinji HIGA, Haruhiko HIRATA, Seigou MINAMI, Shouji HASHIMOTO, Masaki SUEMURA, Yukihiko SAEKI, Ichiro KAWASE, Toshio TANAKA

Author information

Shinji HIGA
The Third Department (Molecular Medicine) of Internal Medicine, Osaka University Medical School
Haruhiko HIRATA
The Third Department (Molecular Medicine) of Internal Medicine, Osaka University Medical School
Seigou MINAMI
The Department of Internal Medicine, Osaka Police Hospital
Shouji HASHIMOTO
The Department of Internal Medicine, Toneyama National Hospital
Masaki SUEMURA
The Third Department of Internal Medicine, Nissay Hospital
Yukihiko SAEKI
The Third Department (Molecular Medicine) of Internal Medicine, Osaka University Medical School
Ichiro KAWASE
The Third Department (Molecular Medicine) of Internal Medicine, Osaka University Medical School
Toshio TANAKA
The Third Department (Molecular Medicine) of Internal Medicine, Osaka University Medical School

Keywords: acquired angioedema, autoantibody, C1 INH

JOURNAL FREE ACCESS

2002 Volume 41 Issue 5 Pages 398-402

DOI https://doi.org/10.2169/internalmedicine.41.398

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Abstract

A 51-year-old man with recurrent episodes of angioedema was diagnosed as having autoimmune acquired angioedema, based on adult onset, lack of apparent family history, decreased activity of C1 esterase inhibitor (C1 INH) and CH50, decreased levels of serum C4 and C1q and the presence of autoantibodies to C1 INH. The danazol treatment relieved the symptoms of angioedema and increased the C1 INH activity and concentration with the normalization of CH50, C1q and C4 levels. To our knowledge, this is the first case of autoimmune acquired angioedema in Japan.
(Internal Medicine 41: 398-402, 2002)

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