Classification and Treatment of Angioedema without Wheals: A Spanish Delphi Consensus

Abstract

Introduction Problems in the definition and classification of angioedema, leading to difficulties in its diagnosis and treatment, have been identified; therefore, an improvement in the current classification of angioedema is required. Objective The aim of this study was to propose a practical classification of angioedema without wheals that helps to establish a differential diagnosis and take appropriate therapeutic decisions. Methods An initial proposal of classification of angioedema without wheals was agreed by a scientific committee of experts and was subsequently validated by a panel of experts by means of consensus based on the Delphi methodology. Forty-five items on the classification, diagnosis, and treatment of angioedema without wheals were proposed for the survey. Results Most items (93.8%) were agreed after two rounds. All panelists agreed with the proposed classification, as well as with most of the clinical and treatment characteristics. The angioedema without wheals classification established three groups: histamine-mediated, bradykinin-mediated, and unknown mechanism angioedema. The clinical characteristics of the proposed types of angioedema were also agreed, except for the allergic histamine-mediated and unknown mechanism angioedema, which generated debate. Regarding treatments, although there was broad agreement with the proposed items, a lack of knowledge about some treatments in this pathology was observed. Conclusion The proposed classification of angioedema without wheals was accepted with a high degree of agreement; however, knowledge of available treatments needs to be increased and the definition of angioedema of unknown mechanism needs to be improved. Supplementary Information The online version contains supplementary material available at 10.1007/s40257-022-00735-7.

Medical algorithm: Diagnosis and management of histaminergic angioedema

“…Depending on the mediator involved, it is classified as either bradykinin‐mediated or histamine‐mediated. The designation of histaminergic angioedema or idiopathic histaminergic acquired angioedema 2 could lead one to think that it has to respond to antihistamines, but this is not the case 3 . The term mast cell‐mediated angioedema (MC‐AE) would be more appropriate to include patients for whom antihistamine therapy is insufficient.…”