Symptom assessment in hypereosinophilic syndrome: Toward development of a patient-reported outcomes tool

Kovacs, Nicholas C.; Benjamin, Katy; Holland-Thomas, Nicole; Moshkovich, Olga; Nelsen, Linda; Ortega, Héctor; Schwartz, E.; Steinfeld, Jonathan; Kirby, Suyong Yun; Klion, Amy D.; Khoury, Paneez

doi:10.1016/j.jaip.2020.04.069

Cited by 8 publications

(8 citation statements)

References 7 publications

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“…Pulmonary involvement presents most frequently with ground-glass opacities, pleural effusions and parenchymal infiltrates which is in line with our case 16. Hepatomegaly, as was observed in our case, has been described in six previous case series according to a recent report 17. In addition, cardiac symptoms at first presentation of HES were only present in less than 5% of the cases 15.…”

Section: Discussionsupporting

confidence: 90%

“…Therefore, it has been suggested to perform echocardiography in all patients diagnosed with HES due to the significant impact of cardiac involvement 18 19. Finally, HES has a significant social and physical impact for patients, as was also described by our patient 17…”

Section: Discussionsupporting

confidence: 57%

“…This illustrates the high heterogeneity of the disease in organ involvement, presenting symptoms and course of disease. In addition, two retrospective studies including larger numbers of patients with HES conclude that dermatological, pulmonary and gastrointestinal symptoms are most frequently involved in HES with generally good response to corticosteroid therapy 9 15–17. Dermatological involvement is present in over 50% of the cases and pulmonary involvement is estimated to be present in approximately 25% of the cases 16.…”

Section: Discussionmentioning

confidence: 99%

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Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up

et al. 2021

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A previously healthy 40-year-old man was referred to our emergency department with pruritic skin lesions and dyspnoea. Laboratory investigation revealed hypereosinophilia. Further diagnostic work-up confirmed the diagnosis of idiopathic hypereosinophilic syndrome (iHES), a rare myeloproliferative disease with a heterogeneous clinical presentation. We describe a unique case with cardiac, pulmonary, hepatic and cutaneous involvement at time of presentation. This case accentuates the importance of an extensive multidisciplinary diagnostic work-up, since iHES is a condition with potential rapid progressive multiorgan failure which requires prompt analysis and treatment. In addition, this case emphasises the importance of being aware of tunnel vision, especially during the COVID-19 pandemic, which might give rise to an increased risk of missing rare diagnoses. Our patient was treated with prednisolone, after which both his clinical condition and eosinophil concentrations markedly improved.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 57%

Section: Discussionmentioning

confidence: 99%

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Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up

et al. 2021

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“…The HES-DS questionnaire (see Supplementary materials) was developed based on a previous qualitative study assessing the symptoms and the impact of HES on patients' health-related quality of life, wherein patients described their symptomatic experience according to organ system (12). The HES-DS questionnaire Abbreviations: eDiary, electronic diary; FIP1L1, Fip1-like 1; HES, hypereosinophilic syndrome; HES-DS, hypereosinophilic syndrome daily symptoms; IV, intravenous; OCS, oral corticosteroids; PDGFRA, platelet-derived growth factor receptor alpha; SC, subcutaneous.…”

Section: Methodsmentioning

confidence: 99%

Mepolizumab therapy improves the most bothersome symptoms in patients with hypereosinophilic syndrome

et al. 2023

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BackgroundHypereosinophilic syndrome (HES) is characterized by persistent elevated blood and/or tissue eosinophil levels and eosinophil-mediated organ damage. Presentation is highly heterogenous; patients may experience symptoms affecting multiple organ systems.ObjectivesTo assess the effects of mepolizumab, which targets interleukin-5, on HES-related symptom burden, based on HES daily symptoms (HES-DS) questionnaire data collected during the Phase III (ClinicalTrials.gov ID: NCT02836496) study of mepolizumab in patients with HES.MethodsEach of the six HES-related symptoms were rated (0–10) daily by patients, recalling worst symptom experience in the prior 24 hours; change from baseline at Week 32 was also calculated for mepolizumab versus placebo.ResultsMepolizumab versus placebo reduced HES-related symptom burden severity in patients with HES at Week 32. Improvements in the median change from baseline scores were seen across all symptom groups except skin for patients treated with mepolizumab; greatest improvement from baseline was observed for breathing symptoms.ConclusionThese data highlight the considerable symptom burden associated with HES and further support the clinical benefits of mepolizumab treatment for these patients.

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“…5 However, the study was limited by its sample size (N=10) and the lack of objective measures for clinical symptom improvement, which is common when studying eosinophilic disorders with a wide range of clinical manifestations such as HES. 5,6 When applied to patients with CRSwNP (N=16), an open-label study of dexpramipexole treatment resulted in significant blood and nasal polyp tissue eosinophil reduction, with the geometric mean absolute eosinophil count (AEC) dropping from 525 cells/μl to 31 cells/μl (p<0.001) and the tissue eosinophils reducing from a mean of 168 cells to 5 cells per high-power field (p=0.001) after 6 months. 7 However, there was no change in total polyp size and, importantly, no significant change in patient nasal symptoms.…”

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confidence: 99%

Dexpramipexole: A Potential Non-biologic Alternative for Patients with Eosinophilic Asthma?

Kuang¹

2022

US Respiratory &Amp; Pulmonary Diseases

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Dexpramipexole offers a potential non-biologic option for patients with eosinophilic asthma in that it lowers blood eosinophil count and improves lung function parameters. However, longer-term studies in patients treated by reducing blood or tissue eosinophils, whether through biologics or oral therapies, are needed to better understand the role of the eosinophil in human biology and disease pathogenesis and to better delineate the clinical efficacy of Dexpramizole in asthma.

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Symptom assessment in hypereosinophilic syndrome: Toward development of a patient-reported outcomes tool

Cited by 8 publications

References 7 publications

Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up

Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up

Mepolizumab therapy improves the most bothersome symptoms in patients with hypereosinophilic syndrome

Dexpramipexole: A Potential Non-biologic Alternative for Patients with Eosinophilic Asthma?

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