6533b870fe1ef96bd12cfcf5

RESEARCH PRODUCT

Recurrent angioedema and the threat of asphyxiation.

subject

description

Angioedema (also known as Quincke disease) is the name given to edema lasting 1–7 days that recurs at irregular intervals. Target organs are the skin, tongue, glottis and larynx, gastrointestinal tract, and sometimes other soft organs. The clinical symptom referred to as angioedema forms part of a variety of disease entities (Box 1, Figure 1). In Germany, according to the present author’s estimate, several thousand patients suffer from one of the forms of recurrent angioedema. Cases of sudden asphyxiation are rare, but do occur every now and again (1). This review aims to draw attention to the various clinical features of recurrent angioedema and the practical steps for dealing with it, and to report the most recent developments in this field. The literature search was carried out on PubMed (search terms: angioedema, C1-inhibitor deficiency). Box 1 Forms of angioedema with and without C1 inhibitor deficiency Hereditary angioedema due to C1 inhibitor deficiency Type 1 (reduced activity and plasma concentration of C1 inhibitor) Type 2 (reduced activity with normal or increased plasma concentration of C1 inhibitor) Hereditary angioedema with normal C1 inhibitor Hereditary angioedema due to mutation of the factor XII gene Hereditary angioedema of unknown genetic cause Angioedema due to acquired C1 inhibitor deficiency Angioedema triggered by ACE inhibitors or other medical drugs Recurrent angioedema in patients with chronic urticaria Recurrent idiopathic angioedema Angioedema as part of an allergic or pseudoallergic reaction Figure 1 Flow diagram for the diagnosis of recurrent angioedema