Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

What causes diffuse idiopathic pulmonary neuroendocrine cell hyperplasia?

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.

Last updated on 05-01-20

How is diffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed?

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.

Last updated on 05-01-20

How might diffuse idiopathic pulmonary neuroendocrine cell hyperplasia be treated?

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. In very severe forms of the condition, palliative lung resection or even lung transplantation may be considered.

Last updated on 05-01-20

Name: Neuroendocrine Tumor Research Foundation NETRF 321 Columbus Avenue, Suite 5R
Boston, MA, 02116 , United States
Phone: +1-617-946-1780 Email: info@netrf.org Url: https://netrf.org/

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