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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

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Other Names:
DIPNECH
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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare and poorly understood lung condition that is characterized by the abnormal overgrowth of certain cells in the lung (called pulmonary neuroendocrine cells) that receive signals from nerve cells (neurons) and produce hormones. People with this diagnosis may have no obvious symptoms or may exhibit features of airway disease such as a chronic, nonproductive cough, shortness of breath with exertion, and wheezing. It is considered to be a precancerous condition as studies suggest it is a precursor for pulmonary carcinoid tumors. The cause of DIPNECH is currently unknown.[1][2] Because so few cases have been reported in the medical literature, there is limited information on the prognosis and management of this condition.[3][4]
Last updated: 7/20/2017
People with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) usually do not have any signs and symptoms of lung disease, but they do have characteristic findings when their lung tissue is examined under a microscope.[1] 

When present, symptoms commonly shared by people with DIPNECH include:[1][2][3]
  • Chronic, nonproductive cough
  • Shortness of breath with exertion
  • Wheezing
Lung function either remains stable or slowly becomes worse over time. However, a small subset of people may have a rapidly progressive and life-threatening disease course.[1]
Last updated: 7/20/2017
The cause of DIPNECH is still unknown. During fetal development, pulmonary neuroendocrine cells (PNC) are located throughout the whole respiratory tract as they have a key role in the regulation of lung development. In adulthood, PNCs are typically less numerous. However, a rapid increase in the number of PNCs (also known as reactive PNEC hyperplasia) may be observed in cigarette smokers, people living in high altitudes, or in people with certain chronic lung conditions (such as asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis, bronchiectasis, and diffuse interstitial pulmonary fibrosis). In contrast to reactive PNEC hyperplasia, people who are affected by DIPNECH do not have any of the risk factors or predisposing conditions listed above.[4]
Last updated: 7/20/2017
According to the World Health Organization (WHO), the diagnosis of DIPNECH is purely based on specific lung tissue characteristics observed under a microscope (i.e., an overgrowth of certain cells in the lung called pulmonary neuroendocrine cells). Lung tissue is obtained with a surgical lung biopsy. The diagnosis may first be suspected based on symptoms and/or a concerning chest imaging study. In other cases, the condition may be diagnosed incidentally in people with no obvious signs of airway disease.[1][4]
Last updated: 7/20/2017
Due to the rarity of the condition, there are no well-established guidelines for the treatment and management of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Several medications have been studied as potential treatments for DIPNECH. Examples include somatostatin analogs (such as octreotide), inhaled corticosteroids, and short-acting beta agonists. Although some people with this disease reported temporary improvement in respiratory symptoms with the use of certain medications, none of the treatments were shown to be beneficial in all patients or to improve the overall outcome.[3] In very severe forms of the condition, palliative lung resection or even lung transplantation may be considered.[4]
Last updated: 7/20/2017

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  1. Rossi G, Cavazza A, Spagnolo P, Sverzellati N, Longo L, Jukna A, Montanari G, Carbonelli C, Vincenzi G, Bogina G, Franco R, Tiseo M, Cottin V, Colby TV. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome. Eur Respir J. June 2016; 47(6):1829-1841. http://erj.ersjournals.com/content/47/6/1829.
  2. Marchevsky AM, Walts AE. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH). Semin Diagn Pathol. November 2015; 32(6):438-444. https://www.ncbi.nlm.nih.gov/pubmed/26472691.
  3. Wirtschafter E, Walts AE, Liu ST, Marchevsky AM. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence. Lung. 2015 Oct;193(5):659-67. October 2015; 193(5):659-667. https://www.ncbi.nlm.nih.gov/pubmed/26104490.
  4. Chassagnon G, Favelle O, Marchand-Adam S, De Muret A, Revel MP. DIPNECH: when to suggest this diagnosis on CT. Clin Radiol. March 2015; 70(3):317-325. https://www.ncbi.nlm.nih.gov/pubmed/25465294.