The diagnosis and treatment of hereditary angioedema patients in Japan: A patient reported outcome survey

• Iwamoto Kazumasa

  Department of Dermatology, Graduate School of Biomedical and Health Sciences, Hiroshima University HAEJ Registered NPO Iwamoto Dermatology & Allergology Clinic

• Yamamoto Beverley

  HAEJ Registered NPO Graduate School of Human Sciences, Osaka University HAEi

• Ohsawa Isao

  HAEJ Registered NPO Department of Nephrology, Internal Medicine, Saiyu Soka Hospital Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Honda Daisuke

  HAEJ Registered NPO Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine

• Horiuchi Takahiko

  Department of Internal Medicine, Kyusyu University Beppu Hospital

• Tanaka Akira

  Department of Oral and Maxillofacial Surgery, School of Life Dentistry at Niigata, The Nippon Dental University

• Fukunaga Atsushi

  Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine

• Maehara Junichi

  Department of Acute Care & General Medicine, Saiseikai Kumamoto Hospital

• Yamashita Kouhei

  Department of Hematology, Kyoto University Hospital

• Akita Tomoyuki

  Department of Epidemiology, Infectious Disease Control and Prevention, Graduate School of Biomedical and Health Sciences, Hiroshima University

• Hide Michihiro

  Department of Dermatology, Graduate School of Biomedical and Health Sciences, Hiroshima University HAEJ Registered NPO

抄録

Background: The rate at which patients are accurately diagnosed with hereditary angioedema (HAE), as well as diagnosed patients access to modern treatments differs greatly among countries. Moreover, the severity and burden of HAE on patients have been reported mostly on the basis of physician-reported surveys. To gain insight into the real-world conditions of patients with HAE through a patient-reported survey in Japan and identify any unmet needs. Methods: A questionnaire was distributed to 121 patients with HAE via a Japanese HAE patient organization during 2016–2017. Responses were collected from 70 patients (57.9%) and subjected to analysis. Results: The average periods from the initial appearance of symptoms (e.g. edema) to a HAE diagnosis was 15.6 years (min–max, 0–53). Patients visited an average of 4.6 different departments until receiving a definitive diagnosis. The average age at the first visit was 25.6 years (3–73) and at diagnosis 32.8 years (0–73). Patients reported an average of 15.7 (0–100) attacks per year, but only 53.1% of attacks were treated. The days of hospitalization due to severe attacks was 14.3 (0–200) before diagnosis, but these declined to 4.3 (0–50) after diagnosis. In the treatment for attacks, 82% of the patients were treated with the plasma-derived C1 inhibitor concentrate, and 69% of the patients reported experiencing a therapeutic effect. Conclusions: There is a long gap between first attack and diagnosis of HAE, and the number of non-treated attacks is high in Japan. Steps are needed to improve the diagnostic and treatment environments to address these issues.

収録刊行物

• Allergology International

  Allergology International 70 (2), 235-243, 2021

  一般社団法人日本アレルギー学会

キーワード

• Consultation behavior
• Hereditary angioedema
• Japan
• Patient survey
• QoL

詳細情報

• CRID: 1390006210126191104
• NII論文ID: 130008024314
• DOI: 10.1016/j.alit.2020.09.008
• ISSN: 14401592
• ISSN: 13238930
• Web Site: https://api.elsevier.com/content/article/PII:S1323893020301350?httpAccept=text/xml
• Web Site: https://api.elsevier.com/content/article/PII:S1323893020301350?httpAccept=text/plain
• Web Site: https://search.jamas.or.jp/link/ui/2022048998
• 本文言語コード: en
• データソース種別:
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