Assessment of disease activity and quality of life in patients with recurrent bradykinin-mediated versus mast cell-mediated angioedema

Pelin Kuteyla Can,Ece Nur Degi̇Rmentepe,Piril Etikan,Kübra Kiziltaç,Asli Gelincik,Semra Demir,Suna Buyukozturk,Eda Haşal,Emel Bülbül Başkan,Ömür Aydin,Marcus Maurer,Karsten Weller,Emek Kocaturk

doi:10.1016/j.waojou.2021.100554

Abstract

ObjectiveRecurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE. MethodsTurkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18–72 years). Patients’ global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE. ResultsTurkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 ± 10.7 vs. 40.7 ± 13.3 years), had longer disease duration (236 ± 178 vs. 51 ± 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 ± 20 vs. 40 ± 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 ± 24.6 vs 55.2 ± 52.9), but AE-QoL was higher (44.2 ± 16.1 vs 34.5 ± 22.5) in BMAE patients as compared to MMAE patients. ConclusionsPatients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.

Highlights

Recurrent angioedema (RAE) is the reoccurrence of localized deep dermal, subcutaneous, or submucosal edema resulting from increased vascular permeability and extravasation for longer than 6 weeks
Recurrent mast cell mediated angioedema; (MMAE) most commonly occurs due to chronic urticaria (CU), either chronic spontaneous urticaria (CSU) or chronic inducible urticaria (CIndU), with or without wheals
The AAS28 scores showed strong correlations with the number of days affected by RAE (r 1⁄4 0.844; p < 0.001) and strong correlations with the PatGA-DA-VRS (r 1⁄4 0.59; p < 0.001) and the PatGA-DA-VAS (r 1⁄4 0.66; p < 0,001)

Summary

Introduction

Recurrent angioedema (RAE) is the reoccurrence of localized deep dermal, subcutaneous, or submucosal edema resulting from increased vascular permeability and extravasation for longer than 6 weeks. Acute MMAE occurs in anaphylaxis, with or without wheals,[4] through an allergic mechanism, type I hypersensitivity, leading to the activation and degranulation of mast cells and their release of mediators such as histamine and leukotrienes.[4,5] Acute forms of MMAE can be caused by nonallergic non-IgE-mediated responses, in response to medications such as nonsteroidal anti-inflammatory drugs (NSAIDs) or infections.[6] Recurrent MMAE most commonly occurs due to chronic urticaria (CU), either chronic spontaneous urticaria (CSU) or chronic inducible urticaria (CIndU), with or without wheals In case of the latter, MMAE is sometimes referred to as idiopathic histaminergic acquired angioedema.[6]

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