Cough in hypereosinophilic syndrome: case report and literature review

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Abstract

Background

Cough and airway eosinophilic inflammation has not been highlighted in hypereosinophilic syndrome (HES).

Case presentation

We report 2 further cases and reviewed the clinical features and treatment of HES present with cough from the literature. Both cases were middle age male, presenting with chronic cough, airway eosinophilic inflammation and hyper eosinophilia who have been previous misdiagnosed as cough-variant asthma and failed anti-asthma treatment. PDGFRA fusion gene was confirmed in one case, but not in the other case. Both had evidence of myeloproliferative features. The tyrosine kinase inhibitor, imatinib, resulted in complete resolution of eosinophilia and cough. By searching PubMed, we found 8 HES cohorts of 411 cases between 1975 and 2013, where the incidence of cough was 23.11%. Sixteen case reports of HES presented with cough as predominant or sole symptom, with nine male patients with positive PDGFRA fusion gene, who responded well to imatinib. Six of seven patients, who tested negative for the PDGFRA, responded to systemic glucocorticoids.

Conclusions

Cough and airway eosinophilic inflammation is common in some HES patients. PDGFRA+ HES patients present with chronic cough respond well to imatinib. Our case reports indicate that PDGFRA negative HES patients may respond to imatinib as well.

Related collections

Most cited references 33

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Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy.

Javed Sheikh, Bruce S. Bochner, Kristin Leiferman … (2009)

Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by persistent blood eosinophilia > or =1.5 x 10(9)/L, absence of a secondary cause, and evidence of eosinophil-associated pathology. With the exception of a recent multicenter trial of mepolizumab (anti-IL-5 mAb), published therapeutic experience has been restricted to case reports and small case series. The purpose of the study was to collect and summarize baseline demographic, clinical, and laboratory characteristics in a large, diverse cohort of patients with HES and to review responses to treatment with conventional and novel therapies. Clinical and laboratory data from 188 patients with HES, seen between January 2001 and December 2006 at 11 institutions in the United States and Europe, were collected retrospectively by chart review. Eighteen of 161 patients (11%) tested were Fip1-like 1-platelet-derived growth factor receptor alpha (FIP1L1-PDGFRA) mutation-positive, and 29 of 168 patients tested (17%) had a demonstrable aberrant or clonal T-cell population. Corticosteroid monotherapy induced complete or partial responses at 1 month in 85% (120/141) of patients with most remaining on maintenance doses (median, 10 mg prednisone equivalent daily for 2 months to 20 years). Hydroxyurea and IFN-alpha (used in 64 and 46 patients, respectively) were also effective, but their use was limited by toxicity. Imatinib (used in 68 patients) was more effective in patients with the FIP1L1-PDGFRA mutation (88%) than in those without (23%; P < .001). This study, the largest clinical analysis of patients with HES to date, not only provides useful information for clinicians but also should stimulate prospective trials to optimize treatment of HES.

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The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.

Michael J. Chusid, Timothy D Dale, Robert West … (1974)

Several closely related disease entities make up the idiopathic hypereosinophilic syndrome (HES). The syndrome is manifest by persistent and prolonged eosinophilia with organ damage. A group of 14 patients had hematologic, cardiac, and neurologic abnormalities attributable to this disease. Patient survival and response to chemotherapy was significantly better in this group than in previously reported patients. The etiology of HES remains unknown, as does the mechanism of tissue damage.

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Chronic cough. The spectrum and frequency of causes, key components of the diagnostic evaluation, and outcome of specific therapy.

R. John Irwin, Gerard Curley, Christopher C. French (1990)

A successful, systematic, anatomic, diagnostic protocol for evaluating patients with chronic cough was presented in 1981. To determine whether it was still valid, we prospectively evaluated, over a 22-month interval, 102 consecutive and unselected immunocompetent patients complaining of cough an average of 53 +/- 97 months (range, 3 wk to 50 yr). Utilizing the anatomic, diagnostic protocol modified to include prolonged esophageal pH monitoring (EPM), the causes of cough were determined in 101 of 102 (99%) patients, leading to specific therapy that was successful in 98%. Cough was due to one condition in 73%, two in 23%, and three in 3%. Postnasal drip syndrome was a cause 41% of the time, asthma 24%, gastroesophageal reflux (GER) 21%, chronic bronchitis 5%, bronchiectasis 4%, and miscellaneous conditions 5%. Cough was the sole presenting manifestation of asthma and GER 28 and 43% of the time, respectively. While history, physical examination, methacholine inhalational challenge (MIC), and EPM yielded the most frequent true positive results, MIC was falsely positive 22% of the time in predicting that asthma was the cause of cough. Laboratory testing was particularly useful in ruling out suspected possibilities. We conclude that the anatomic diagnostic protocol is still valid and that it has well-defined strengths and limitations.

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Author and article information

Contributors

Chunyan Wang: wcy163@163.com

Kefang Lai: klai@163.com

Journal

Journal ID (nlm-ta): BMC Pulm Med

Journal ID (iso-abbrev): BMC Pulm Med

Title: BMC Pulmonary Medicine

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2466

Publication date (Electronic): 15 April 2020

Publication date PMC-release: 15 April 2020

Publication date Collection: 2020

Volume: 20

Electronic Location Identifier: 90

Affiliations

[1 ]GRID grid.470124.4, Department of Allergy and Clinical Immunology, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Diseases, Guangzhou Institute of Respiratory Health, , The First Affiliated Hospital of Guangzhou Medical University, ; Guangzhou, China

[2 ]ISNI 0000 0001 2113 8111, GRID grid.7445.2, National Heart and Lung Institute, Imperial College London & Royal Brompton Biomedical Research Unit at Royal Brompton and Harefield NHS Foundation Trust, ; London, UK

[3 ]GRID grid.470124.4, Department of hematology, , The First Affiliated Hospital of Guangzhou Medical University, ; 1 Kangda Road, Guangzhou, 510230 China

[4 ]GRID grid.470124.4, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, , the First Affiliated Hospital of Guangzhou Medical University, ; 151 Yanjiang Road, Guangzhou, 510120 China

Article

Publisher ID: 1134

DOI: 10.1186/s12890-020-1134-x

PMC ID: 7158094

PubMed ID: 32293378

SO-VID: 2ff2a437-3799-45b5-8fbb-e89db3dd952c

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 11 October 2019

Date accepted : 3 April 2020

Funding

Funded by: FundRef http://dx.doi.org/10.13039/100009659, Guangzhou Medical University;

Award ID: 500102010501069001

Award Recipient : Kian Fan Chung

Custom metadata

ScienceOpen disciplines: Respiratory medicine

Keywords: airway eosinophilic inflammation,cough,hypereosinophilic syndrome,imatinib,pdgfra fusion gene

Data availability:

ScienceOpen disciplines: Respiratory medicine

Keywords: airway eosinophilic inflammation, cough, hypereosinophilic syndrome, imatinib, pdgfra fusion gene

Cough in hypereosinophilic syndrome: case report and literature review

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Abstract

Background

Case presentation

Conclusions

Related collections

Inflamed lung in vitro co-culture models

Most cited references 33

Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy.

The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.

Chronic cough. The spectrum and frequency of causes, key components of the diagnostic evaluation, and outcome of specific therapy.

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Affiliations

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Funding

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