Diminished capacity of opsonization and immune complex solubilization, and detection of anti-C1q antibodies in sera from patients with hereditary angioedema

• Honda Daisuke

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Ohsawa Isao

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine Nephrology Unit, Saiyu Soka Hospital

• Sato Nobuyuki

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Inoshita Hiroyuki

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Mano Satoshi

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Tomino Yasuhiko

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine Medical Corporation Showakai

• Suzuki Yusuke

  Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

抄録

Background: Hereditary angioedema (HAE) is an autosomal dominant disease caused by deficiency of C1 esterase inhibitor. Symptoms of HAE include edema, which can potentially cause suffocation. Some patients with HAE exhibit immunological abnormalities, which could prevent an accurate diagnosis. Low levels of complement components are characteristic of HAE and in other settings are thought to reduce elimination of apoptotic cells and immune complex (IC). Thus, we aimed to experimentally clarify the mechanism of immunological abnormalities using sera from HAE patients. Methods: Serum samples from 18 patients with HAE were collected when free from angioedema attack and compared with normal human pooled sera (NHPS) from 20 healthy volunteers. Opsonization was measured as the rate of phagocytosis of apoptotic Jurkat cells by macrophages differentiated from THP-1 cells incubated with serum. IC solubilization in serum was analyzed by quantifying peroxidase released from a synthetic IC composed of peroxidase and anti-peroxidase antibodies. Anti-C1q antibody levels were detected using an enzyme-linked immunosorbent assay. Results: Serological immunological abnormalities were detected in 12 patients. Opsonization in serum samples from each patient with HAE was lower than that in NHPS (∼20% versus 70%, respectively). The rate of IC solubilization was lower in serum from HAE patients than NHPS. Some patients had high serum anti-C1q antibody levels with increased serum IC levels. Conclusions: Sera from patients with HAE exhibit anti-C1q antibodies, with a lower capacity for opsonization and IC solubilization. This may be associated with immunological abnormalities and should be investigated further to facilitate accurate diagnosis of HAE.

収録刊行物

• Allergology International

  Allergology International 66 (4), 603-609, 2017

  一般社団法人日本アレルギー学会

キーワード

• Anti-C1q antibody
• Autoimmune disease
• Hereditary angioedema
• Immune complex
• Phagocytosis

詳細情報

• CRID: 1390001204632872192
• NII論文ID: 130006179503
• DOI: 10.1016/j.alit.2017.03.008
• ISSN: 14401592
• ISSN: 13238930
• Web Site: https://api.elsevier.com/content/article/PII:S1323893017300412?httpAccept=text/xml
• Web Site: https://api.elsevier.com/content/article/PII:S1323893017300412?httpAccept=text/plain
• Web Site: https://search.jamas.or.jp/link/ui/2018302411
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• 抄録ライセンスフラグ: 使用不可