Episodic Angioedema with Hypereosinophilia (Gleich’s Syndrome): A Case Report and Extensive Review of the Literature

Mormile, Ilaria; Petraroli, Angelica; Loffredo, Stefania; Rossi, Francesca Wanda; Mormile, Mauro; Mastro, A. Del; Spadaro, Giuseppe; Paulis, Amato de; Bova, María

doi:10.3390/jcm10071442

Cited by 12 publications

(8 citation statements)

References 45 publications

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“…Furthermore, four-month eosinophil monitoring can be included in the six-month evaluation period suggested by the GINA guidelines [33,51] for the diagnosis of SBA. Finally, our model could be also used for other diseases related to eosinophilia-for example, in hypereosinophilic syndrome [52][53][54] and eosinophilic esophagitis [55]-to evaluate the trend of eosinophils in response to several factors and therapy.…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

D’Aiuto,

Mormile,

Granata

et al. 2024

JCM

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Background: Eosinophilia can be influenced by multiple factors. This study aims to set a protocol for monitoring blood absolute eosinophil count (AEC) in patients with seasonal allergy affected by bronchial asthma (BA), allergic rhinitis (AR), or chronic rhinosinusitis with or without nasal polyposis (CRSw/sNP). Methods: We planned a total of four annual blood samples to measure AEC in- and out-seasonal pollen exposure (i.e., one measurement every three months for one year). Results: We identified two distinct groups of patients (non-eosinophilic and eosinophilic). Patients in the eosinophilic group presented with four different patterns (episodic, transient, floating, and persistent). Most patients with episodic, transient, and floating patterns were affected by mild allergy and the increase in eosinophils was related to allergen exposure. In contrast, patients with the persistent pattern mostly presented with more severe allergy (i.e., severe BA and relapsing CRSwNP) and the eosinophilia was unrelated to allergen exposure. The subgroup of patients with severe BA, relapsing CRSwNP, and persistent eosinophilc pattern were treated with benralizumab, which induced a noteworthy improvement in both severe BA and CRSwNP. Conclusions: Multiple AEC measurements in patients with seasonal allergy can better reflect patient’s eosinophilic status and help define the relationship of AEC enhancement with allergen exposure.

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Section: Discussionmentioning

confidence: 99%

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

D’Aiuto,

Mormile,

Granata

et al. 2024

JCM

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“…Systemic hypereosinophilic diseases including eosinophilic granulomatosis with polyangiitis (EGPA) can also be associated with airway inflammation and severe asthma [ 119 , 128 , 129 ]. An early identification of these disabling diseases is essential as they require a multidisciplinary approach and systemic corticosteroids and/or immune-suppressant therapies.…”

Section: Eosinophilic Airway Inflammation and Comorbiditiesmentioning

confidence: 99%

Eosinophilic Airway Diseases: From Pathophysiological Mechanisms to Clinical Practice

Mormile

Fuschillo

et al. 2023

IJMS

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Eosinophils play a key role in airway inflammation in many diseases, such as allergic and non-allergic asthma, chronic rhinosinusitis with nasal polyps, and chronic obstructive pulmonary disease. In these chronic disabling conditions, eosinophils contribute to tissue damage, repair, remodeling, and disease persistence through the production a variety of mediators. With the introduction of biological drugs for the treatment of these respiratory diseases, the classification of patients based on clinical characteristics (phenotype) and pathobiological mechanisms (endotype) has become mandatory. This need is particularly evident in severe asthma, where, despite the great scientific efforts to understand the immunological pathways underlying clinical phenotypes, the identification of specific biomarkers defining endotypes or predicting pharmacological response remains unsatisfied. In addition, a significant heterogeneity also exists among patients with other airway diseases. In this review, we describe some of the immunological differences in eosinophilic airway inflammation associated with severe asthma and other airway diseases and how these factors might influence the clinical presentation, with the aim of clarifying when eosinophils play a key pathogenic role and, therefore, represent the preferred therapeutic target.

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“…Episodic angioedema with hypereosinophilia (Gleich's syndrome) 79 Weight gain, fever, pruritus, sometimes urticaria Cluster headache 80 Presence of headache, conjunctival injection, ptosis, pupil constriction, watering of eyes Idiopathic edema 81 Pitting edema, periorbital edema after recumbency overnight, weight gain from morning to evening…”

Section: Condition Differential Diagnosismentioning

confidence: 99%

Could it be hereditary angioedema?—Perspectives from different medical specialties

Magerl,

Sala‐Cunill,

Weber‐Chrysochoou

et al. 2023

Clinical & Translational All

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Hereditary angioedema (HAE) is a rare autosomal dominant disease, with patients often suffering with associated symptoms for many years before receiving a correct diagnosis. The symptoms greatly impact a patient's quality of life (QoL) and include excruciating abdominal pain and angioedema of the skin and submucosa. Angioedema of the larynx represents a significant mortality risk in undiagnosed patients, and a large proportion of patients with HAE receive incorrect diagnoses and undergo unnecessary surgery. HAE‐specific treatments can control and prevent acute life‐threatening episodes, in addition to improving QoL, emphasizing the value of early diagnosis for patients. Diagnostic delay may be due to a lack of HAE awareness by healthcare professionals and the similarity of HAE symptoms with those of more common conditions, complicating differential diagnosis. The multifaceted nature of the condition may result in visits to one of many different medical settings, for example: the Emergency Room, pediatrics, general practice, otolaryngology, gastroenterology, and dermatology. Therefore, it is crucial that physicians in multiple healthcare specialties are aware of the disease to ensure that patients with HAE receive a timely diagnosis. Using patient cases from various medical specialties, this review highlights the necessity for cross‐specialty awareness of HAE and outlines the essential information for the various healthcare professionals that may encounter a patient with HAE symptoms, in order to effectively treat and/or diagnose HAE.

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Episodic Angioedema with Hypereosinophilia (Gleich’s Syndrome): A Case Report and Extensive Review of the Literature

Cited by 12 publications

References 45 publications

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

Eosinophilic Patterns in Patients with Seasonal Allergy Affected by Bronchial Asthma and Rhinitis/Rhinosinusitis: Efficacy of Benralizumab in Patients with the Persistent Pattern

Eosinophilic Airway Diseases: From Pathophysiological Mechanisms to Clinical Practice

Could it be hereditary angioedema?—Perspectives from different medical specialties

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