The acquired angioedema – a bi-center retrospective study on diagnosis, course and therapy

Introduction Bradykinin mediated angioedemas are less common than mastcell mediated angioedemas. Not only the genetically determined hereditary angioedema (HAE), but also the rare acquired angioedema (AAE) may cause low levels of C1-inhibitor (C1-INH). Finding and curing the underlying - often malignant – condition is the only approved contemporary therapy.

Methods The data of all AAE patients referring to two university medical centers in southern Germany was analyzed retrospectively. All the available information was evaluated in terms of diagnosing the AAE as well as the underlying condition, laboratory parameters and effectiveness of off-label therapy.

Results 16 patients with AAE have been included. Symptom onset was at a mean age of 62,3 years (range 43 – 80 years). Swellings of the face were the most common, followed by abdominal and peripheral edemas. None suffered from a laryngeal attack. The vast majority of patients showed low values for C1-INH levels, activity, and C4 levels, 25% of patients showed normal C1q levels. A hematological disorder was found in half of the cases. The off-label acute therapy consisted of Icatibant or C1-INH concentrate, in singular cases prophylactic treatment was given with C1-INH concentrate.

Conclusion Though laryngeal attacks appear to be rare in cases of AAE, the correct diagnosis of AAE can be life-saving for patients. This includes regular haemato-oncological care. Along with patients’ history and therapy success, laboratory findings give proof of the AAE in most cases. Options for acute treatment and eventually prophylaxis for severe cases are essential despite the lack of approval, especially when the underlying disease is not currently known or not in need of treatment.

Publication History

Article published online:
24 May 2022