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Hypereosinophilic obliterative bronchiolitis: a distinct, unrecognised syndrome

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Published:25th Mar 2020
Background: Only isolated biopsy-proven cases of eosinophilic bronchiolitis have been reported, all from Japan. Methods: We present 6 patients with hypereosinophilic obliterative bronchiolitis (HOB), defined by the following criteria: 1-blood eosinophil cell count>1 G�L?1 and/or BAL eosinophil count>25%, 2-persistent airflow obstruction despite high-dose inhaled bronchodilators and corticosteroids, 3-eosinophilic bronchiolitis at lung biopsy (n=1) and/or direct signs of bronchiolitis (centrilobular nodules, branching opacities) on computed tomography (n=6). Results: Chronic dyspnoea and cough often severe, without the characteristic features of asthma, were the main clinical manifestations. Atopy and asthma were present in the history of 3 and 2 patients, respectively. One patient met biological criteria of the lymphoid variant of idiopathic hypereosinophilic syndrome. Mean blood eosinophil cell count was 2.7 G�L?1 and mean eosinophil differential percentage at bronchoalveolar lavage was 63%. Mean initial FEV1/FVC ratio was 50%, normalising with oral corticosteroid therapy in all patients. HOB manifestations recurred when oral prednisone was decreased to 10�20 mg�day?1, but higher doses controlled the disease. Conclusion: HOB is a characteristic entity deserving to be individualised among the eosinophilic respiratory disorders. Thorough analysis is needed to determine whether unrecognised and/or smouldering HOB may further be a cause of irreversible airflow obstruction in chronic eosinophilic respiratory diseases.

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