Diffuse idiopathic neuroendocrine cell hyperplasia as a rare cause of chronic cough.

Research paper by Paula Inês PI Pedro, Dolores D Canário, Miguel M Lopes, Ana A Oliveira

Indexed on: 21 Dec '18Published on: 21 Dec '18Published in: BMJ case reports


A 39-year-old Caucasian woman, who has never smoked, presented a 16-year-duration chronic dry cough. She was prescribed by her general physician with corticosteroid and long-acting β-agonist inhalers assuming it was asthma, with mild symptomatic improvement. When cough got more persistent and associated with exertional dyspnoea and wheezing, a chest CT scan was performed, which showed multiple bilateral micronodular formations and diffuse mosaic lung pattern with air trapping. She was sent to our Respiratory Department and performed a bronchoalveolar lavage and cryobiopsy that were inconclusive. She underwent surgical lung biopsy with pathology revealing multiple foci of neuroendocrine cell hyperplasia and tumourlets associated with constrictive bronchiolitis, a histological pattern suggestive of diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH). DIPNECH is a rare and preinvasive disease. Presenting symptoms can be cough and breathlessness. At the time of writing, the patient is on octreotide with symptomatic improvement. © BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.