Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz

Logo MUG-Forschungsportal

Gewählte Publikation:

SHR Neuro Krebs Kardio Lipid

Zanichelli, A; Longhurst, HJ; Maurer, M; Bouillet, L; Aberer, W; Fabien, V; Andresen, I; Caballero, T; IOS Study Group.
Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical setting.
Ann Allergy Asthma Immunol. 2016; 117(4):394-398 [OPEN ACCESS]
Web of Science PubMed FullText FullText_MUG


Autor/innen der Med Uni Graz:
Aberer Werner

Dimensions Citations:

Plum Analytics:
Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) causes swelling in the skin and upper airways and pain in the abdomen because of mucosal swelling. C1-INH-HAE is frequently misdiagnosed, leading to delays in diagnosis, inadequate treatment, and unnecessary procedures. To evaluate the history of misdiagnosis in patients participating in the Icatibant Outcome Survey (IOS). The IOS is an observational study in which safety and effectiveness of icatibant have been evaluated since 2009. As part of the IOS, patients record any misdiagnoses received before being diagnosed as having C1-INH-HAE. In January 2016, a total of 418 of 633 IOS patients with C1-INH-HAE type I or II had provided misdiagnosis data. Of these, 185 of 418 (44.3%) received 1 or more prior misdiagnoses. The most common misdiagnoses were allergic angioedema (103 of 185) and appendicitis (50 of 185). A variety of other misdiagnoses were reported, including a substantial number of gastrointestinal disorders (excluding appendicitis). Misdiagnosis rates were similar between males (41.1%) and females (46.5%) and between C1-INH-HAE type I (43.7%) and type II (51.6%). Patients with family members diagnosed as having C1-INH-HAE were significantly less likely to be misdiagnosed than patients without a family history (140 of 366 [41.7%] vs 38 of 58 [65.5%], respectively; P = .001). Patients with a prior misdiagnosis had longer median delay to C1-INH-HAE diagnosis (13.3 years) than patients without (1.7 years; P < .001). From this large database, approximately 50% of patients with C1-INH-HAE type I or II have previously had their conditions misdiagnosed, most commonly as allergic angioedema or appendicitis. Misdiagnosis results in marked delays in receiving the correct diagnosis, during which time patients cannot access effective, lifesaving treatment. NCT01034969. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.
Find related publications in this database (using NLM MeSH Indexing)
Adolescent -
Adult -
Aged -
Aged, 80 and over -
Angioedemas, Hereditary - diagnosis
Anti-Inflammatory Agents, Non-Steroidal -
Bradykinin - analogs & derivatives
Child -
Child, Preschool -
Diagnostic Errors - trends
Female -
Humans -
Infant -
Male -
Middle Aged -
Young Adult -

© Meduni Graz Impressum