Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema

Noémi Andrási, Nóra Veszeli, Kinga Viktória Kőhalmi, Dorottya Csuka, György Temesszentandrási, Lilian Varga, Henriette Farkas

Research output: Contribution to journalArticle

4 Citations (Scopus)


Background: The mechanism of idiopathic nonhistaminergic acquired angioedema (InH-AAE) has not yet been precisely elucidated. This condition is characterized by recurrent angioedema without wheals. Objective: To study the clinical features of InH-AAE, and to make, for the first time, independent comparisons with hereditary angioedema of unknown origin (U-HAE), as well as with hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE). Methods: We compared the clinical parameters of 46 patients with InH-AAE with those of 27 patients suffering from U-HAE, as well as of 73 patients with C1-INH-HAE. Results: The mean age at the onset of symptoms was 36 years in InH-AAE, 13 years in C1-INH-HAE, and 29 years in U-HAE. More than 12 edematous episodes occurred over a year in 56% of patients with InH-AAE, in 59% of those with C1-INH-HAE, and in 48% of those with U-HAE. Edema of the extremities, of the upper airways, and of the gastrointestinal tract was more common in patients with C1-INH-HAE (92%, 51%, and 75%, respectively). These manifestations occurred less frequently in patients with InH-AAE (54%, 28%, and 20%) and in patients with U-HAE (37%, 29%, and 20%). By contrast, facial edema occurred in only 15% of patients with C1-INH-HAE, but in 67% of patients with InH-AAE and in 59% of patients with U-HAE. Conclusions: The clinical manifestations of patients with InH-AAE were different from those of patients with C1-INH-HAE. This may indicate different processes underlying edema formation in these disease forms. The close resemblance of the clinical manifestations in InH-AAE and U-HAE might suggest a similarity between the pathophysiology of these conditions.

Original languageEnglish
Pages (from-to)1205-1208
Number of pages4
JournalJournal of Allergy and Clinical Immunology: In Practice
Issue number4
Publication statusPublished - Jul 1 2018


  • Angioedema without wheals
  • Angioedematous attack
  • Clinical characteristic
  • Hereditary angioedema
  • Idiopathic angioedema
  • Nonhistaminergic angioedema

ASJC Scopus subject areas

  • Immunology and Allergy

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