AI Article Synopsis

  • Idiopathic histaminergic acquired angioedema (IH-AAE) is a frequent cause of recurring angioedema without wheals, linked to mast cell activity and related to chronic urticaria (CU).
  • A study identified 31 patients (15 men and 16 women) averaging 50 years old, who experienced an average diagnosis delay of 6.3 years, with 38.7% having a history of allergies.
  • Attacks lasted about 28.1 hours, primarily affected the upper respiratory tract, and often required higher than recommended doses of anti-histamines for symptom control, indicating a benign yet challenging condition.

Article Abstract

Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4908297PMC
http://dx.doi.org/10.1111/cei.12789DOI Listing

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