title: Clinical similarities among bradykinin-mediated and mast cell-mediated subtypes of non-hereditary angioedema: a retrospective study.
abstract: BACKGROUND
  Non-hereditary angioedema (non-HAE) is characterized by local swelling due to self-limiting, subcutaneous or submucosal extravasation of fluid, and can be divided into three subtypes. These subtypes are believed to have different pathophysiological backgrounds and are referred to in recent guidelines as bradykinin-mediated (e.g. caused by angiotensin-converting-enzyme-inhibitors), mast cell-mediated (e.g. angioedema with wheals) or idiopathic (cause unknown). Bradykinin-mediated subtypes are more closely related to hereditary angioedema than the other forms. Because clinical features of these non-HAE subtypes have not been studied in detail, we have looked at the clinical characteristics of symptoms and potential differences in clinical presentation of bradykinin-mediated and mast cell-mediated angioedema (AE) subtypes.
  
  
  METHODS
  A questionnaire was sent to patients presenting with AE at our tertiary outpatient clinic to document clinical characteristics, potential triggers and location of AE. The severity of AE attacks was analysed using visual analogue scales (VAS).
  
  
  RESULTS
  The questionnaire was returned by 106 patients, of which 104 were included in the analysis. AE with wheals, idiopathic AE, and drug-associated AE occurred in 64 (62%), 25 (24%) and 15 patients (14%) respectively. Most patients (62%) reported prodromal symptoms while 63% reported multiple locations for an attack. Face and oropharynx were the main locations of AE attacks of any subtype while swelling was the symptom most frequently reported as severe. Overall severity of the last attack was indicated as severe by 68% of the patients. There were no differences between the subgroups.
  
  
  CONCLUSIONS
  This similarity in clinical presentation raises the possibility that ACEi-induced, mast cell-mediated and idiopathic AE share common pathways.
authors: Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, Utrecht, 3508 GA The Netherlands.
authors: Karlijn J G
authors: KJG
authors: Schulkes
authors: Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, Utrecht, 3508 GA The Netherlands.
authors: Mignon T
authors: MT
authors: Van den Elzen
authors: Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, Utrecht, 3508 GA The Netherlands.|Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: Erik C
authors: EC
authors: Hack
authors: Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: Henderikus G
authors: HG
authors: Otten
authors: Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, Utrecht, 3508 GA The Netherlands.
authors: Carla A F M
authors: CAFM
authors: Bruijnzeel-Koomen
authors: Department of Dermatology and Allergology, University Medical Center Utrecht (G02.124), PO Box 85.500, Utrecht, 3508 GA The Netherlands.
authors: André C
authors: AC
authors: Knulst
keywords: Angioedema; Drug-associated; Idiopathic; Wheals