[Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)]

Abstract

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare, chronic condition that predominantly affects women over the age of 60.

Current knowledge: DIPNECH combines non-specific clinical signs (chronic cough, dyspnea), bronchial obstruction on PFT and signs suggestive of bronchiolitis on chest CT associated with nodules and multiple micronodules. The diagnosis is most often histological, associating neuroendocrine cell hyperplasia, tumorlets and, inconsistently, carcinoid tumors and constrictive bronchiolitis.

Prospects: There are currently no recommendations for DIPNECH treatment and the literature is limited to case reports and retrospective series. Inhibitors of mTOR and somatostatin analogs are possible treatments requiring validation by clinical trials. Functional follow-up and CT scan monitoring are necessary in order to detect complications.

Conclusions: DIPNECH is a rare, usually insidious pathology, and may in some cases expose the patient to a risk of tumor and chronic respiratory failure. The diagnosis should be made in the event of a chronic cough in a middle-aged woman.

Keywords: Bronchiolite; Bronchiolitis; Carcinoid tumor; Chronic cough; DIPNECH; Hyperplasie neuroendocrine; Neuroendocrine hyperplasia; Toux chronique; Tumeur carcinoïde.