A Comparative Study of Sex Distribution, Autoimmunity, Blood, and Inflammatory Parameters in Chronic Spontaneous Urticaria with Angioedema and Chronic Histaminergic Angioedema

Sabaté‐Brescó, Marina; Rodríguez‐Garijo, Nuria; Azofra, Julián; Baeza, M.L.; Donado, Carmen Díaz; Gaig, P.; Guilarte, Mar; Herrera-Lasso, Valeria; Labrador‐Horrillo, Moisés; Sala‐Cunill, Anna; Veleiro, Beatriz; Gil, Maria Pilar; Kaplan, Allen P.; Ferrer, Marta

doi:10.1016/j.jaip.2021.03.038

Cited by 19 publications

(31 citation statements)

References 45 publications

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“…As observed in Table 1 and as previously reported for this cohort, 8 significantly different gender and age distributions were observed between the two groups. The male/female ratio in CHA was 0.78, whereas that in CSU was 0.36.…”

Section: Demographic Characteristicssupporting

confidence: 88%

“…The presence of angioedema in CSU (compared to CSU without angioedema) has not been shown to be associated with altered levels of anti-FceRI, anti-IgE, substance P, B cell activation factor and tryptase, 15 which is consistent with them being two presentations of the same entity. However, CHA does seem to present differences in sex distribution and autoimmunity 8 or the presence of basopenia 8 or eosinopenia. It is very interesting that in this study, D-dimer was inversely correlated with CHA disease activity but not observed for CSU-AE patients.…”

Section: Discussionmentioning

confidence: 86%

“…Disease activity was determined by the following questionnaires: Angioedema Activity Score for 7 consecutive days (AAS7) in both CHA and CSU-AE patients and Urticaria Activity Score over 7 Days (UAS7) in CSU-AE patients. The AAS7 score was categorized into four score levels: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and high (19-105). 17 UAS7 was also divided into four score bands: no episodes (0), low (1)(2)(3)(4)(5)(6), moderate and high (28-42).…”

Section: Patients and Clinical Datamentioning

confidence: 99%

“…7 For this reason and because CHA responds to urticaria treatments, CHA is typically considered a subtype of CSU without wheals. However, a recent study 8 found several features that differentiate CHA from CSU, suggesting that CHA should be considered a separate entity. Angioedema can be disfiguring, limit daily activities and have a significant impact on quality of life.…”

Section: Introductionmentioning

confidence: 98%

“…Angioedema Quality of Life questionnaire by the severity of the attacks on the Angioedema Activity Score. A) Total AE-QoL score in CHA and CSU-AE grouped according to the AAS7 score levels as follows: no episodes (AAS7:0), low (1-6), moderate(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and high (19-105). B) AE-QoL score of the different dimensions in CHA and CSU-AE with patients grouped according to the AAS7 score levels.…”

mentioning

confidence: 99%

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Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Sabaté‐Brescó

Rodríguez‐Garijo

Azofra

et al. 2021

Preprint

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Background: Chronic histaminergic angioedema (CHA) is defined as recurrent episodes of isolated angioedema (without hives) of unknown cause that respond to the same treatment as chronic spontaneous urticaria (CSU). Quality of life (QoL) studies have not been performed for CHA, except those carried out in the context of CSU associated with angioedema attacks (CSU-AE). Moreover, biomarkers for monitoring disease activity in CHA have not been identified. We aim to describe the burden of CHA and impact on patient QoL, compare the findings to those in CSU-AE patients, and investigate biomarker associations with disease severity and QoL parameters. Methods: We performed a prospective multicenter study that included 68 patients with CHA and 63 patients with CSU-AE. Demographic and clinical variables were collected. Validated patient-reported questionnaires were employed to analyze the quality of life and disease activity. Blood and serological parameters, including blood cell count, C-reactive protein, D-dimer and total IgE, were also analyzed. Results: Angioedema disease activity was significantly higher in CSU-AE patients (median AAS7, IQR: 1, [0–1]) than CHA patients (0, [0–1]; p= 0.022). A considerable impact on QoL was found in both groups, although significantly worse values were found for CSU-AE (median AEQoL, IQR: 37, [10–65]; p=0.005). CHA patients were older than CSU-AE patients, and female predominance was not observed. Conclusions: Angioedema severity and QoL impacts are significantly worse in CSU than in chronic histaminergic angioedema. Angioedema should be included in severity urticaria scores (UAS) as well as in specific quality of life urticaria scales.

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Section: Demographic Characteristicssupporting

confidence: 88%

Section: Discussionmentioning

confidence: 86%

Section: Patients and Clinical Datamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 98%

mentioning

confidence: 99%

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Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Sabaté‐Brescó

Rodríguez‐Garijo

Azofra

et al. 2021

Preprint

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Clinical profile of idiopathic angioedema based on severity and treatment response is independent of the presence of concomitant wheals

Soegiharto

Aghdam

Knulst

et al. 2023

JEADV Clinical Practice

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Background Idiopathic angioedema varies in disease severity and treatment response, possibly due to different pathophysiological mechanisms. The presence of wheals is an indicator for histamine‐mediated angioedema. Idiopathic angioedema patients are treated in accordance with chronic spontaneous urticaria guidelines. Little is known about treatment effectiveness in idiopathic angioedema patients without wheals in comparison to idiopathic angioedema patients with concomitant wheals. Objective To describe the disease severity profile in patients with angioedema of unknown cause in relation to prophylactic treatment and the presence or absence of concomitant wheals. Methods In this retrospective cohort study, all records of angioedema patients visiting the outpatient clinic of the UMC Utrecht between January 2015 and March 2020 were screened. Patients with idiopathic angioedema, including those with concomitant wheals, were included. Attack frequency, patient‐reported disease control and attack treatment as indicator for severity were analysed in relation to prophylactic treatment at follow‐up and outcomes were compared between patients with and without concomitant subordinary wheals. Results Two hundred thirty‐six patients were included: 95% (139/236) with angioedema only and 41% (97/236) with angioedema and concomitant subordinary wheals. No prophylactic treatment was prescribed in 27% (64/236), with well‐controlled disease in 86% (25/29) of patients. Antihistamine monotherapy was used in 59% (139/236) of patients and resulted in well‐controlled disease in 68% (62/92). Add‐on treatment was prescribed in 14% (33/236) of patients, omalizumab in 9% (22/236) specifically, with complete response in 38% (6/16) of patients and low attack frequency in another 18% (3/16). Difficult‐to‐treat disease was seen in 8% (18/236), with no response to a fourfold dose of antihistamines or omalizumab. All findings were independent from the presence of concomitant wheals. Conclusion Angioedema is well manageable in the majority of patients without prophylactic therapy or antihistamine monotherapy, but a substantial proportion does not respond to antihistamines and/or omalizumab. Treatment response was independent of the presence or absence of concomitant wheals.

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Classification and Treatment of Angioedema without Wheals: A Spanish Delphi Consensus

2022

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Introduction Problems in the definition and classification of angioedema, leading to difficulties in its diagnosis and treatment, have been identified; therefore, an improvement in the current classification of angioedema is required. Objective The aim of this study was to propose a practical classification of angioedema without wheals that helps to establish a differential diagnosis and take appropriate therapeutic decisions. Methods An initial proposal of classification of angioedema without wheals was agreed by a scientific committee of experts and was subsequently validated by a panel of experts by means of consensus based on the Delphi methodology. Forty-five items on the classification, diagnosis, and treatment of angioedema without wheals were proposed for the survey. Results Most items (93.8%) were agreed after two rounds. All panelists agreed with the proposed classification, as well as with most of the clinical and treatment characteristics. The angioedema without wheals classification established three groups: histamine-mediated, bradykinin-mediated, and unknown mechanism angioedema. The clinical characteristics of the proposed types of angioedema were also agreed, except for the allergic histamine-mediated and unknown mechanism angioedema, which generated debate. Regarding treatments, although there was broad agreement with the proposed items, a lack of knowledge about some treatments in this pathology was observed. Conclusion The proposed classification of angioedema without wheals was accepted with a high degree of agreement; however, knowledge of available treatments needs to be increased and the definition of angioedema of unknown mechanism needs to be improved. Supplementary Information The online version contains supplementary material available at 10.1007/s40257-022-00735-7.

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A Comparative Study of Sex Distribution, Autoimmunity, Blood, and Inflammatory Parameters in Chronic Spontaneous Urticaria with Angioedema and Chronic Histaminergic Angioedema

Cited by 19 publications

References 45 publications

Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Angioedema severity and impacts on quality of life: chronic histaminergic angioedema versus chronic spontaneous urticaria

Clinical profile of idiopathic angioedema based on severity and treatment response is independent of the presence of concomitant wheals

Classification and Treatment of Angioedema without Wheals: A Spanish Delphi Consensus

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