Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Updates to Article Attributes
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is an extremely rare pulmonary disorder where there is a proliferation of neuroendocrine cells within the lung. It is however recognised with increasing frequency.
Epidemiology
There may be an increased female predilection 3. Most patients tend to be non smokers.
Clinical presentation
Most are asymptomatic where discovery is made incidentally on thoracic imaging for an unrelated reason. Some however may present with symptoms such as an insidious cough or a slowly progressive dyspnoea 7.
Pathology
It is characterised by a generalised proliferation of scattered single cells, small nodules or linear proliferations of pulmonary neuroendocrine cells. It is sometimes considered to be a precursor for pulmonary carcinoid tumours.
Radiographic features
CT: HRCT chest
Various features have described each of which are non specific on their own. These include:
- small lung nodules: considered a generally frequent feature 3-4
- ground-glass changes 3
- mosaic perfusion pattern 6-7: due to air trapping
- bronchial wall thickening 7
- mucus plugging
- bronchiectasis 3,7
History and etymology
The first clinical series of six cases describing DIPNECH was published by Aguayo et al in 1992.
Treatment and prognosis
The rarity of the condition poses some clinical challenge on establishing treatment and assessing prognosis. Limited studies suggest that the condition can have a variable prognosis ranging from clinical improvement to being stable to clinical deterioration (rare 5). Various treatment strategies have been described which include systemic and inhaled corticosteroids, bronchodilators, and lung resection.
See also
-<li><a href="/articles/mucus-plugging">mucus plugging</a></li>- +<li><a title="Mucus plugging" href="/articles/mucoid-impaction">mucus plugging</a></li>
-</ul><h4>Treatment and prognosis</h4><p>The rarity of the condition poses some clinical challenge on establishing treatment and assessing prognosis. Limited studies suggest that the condition can have a variable prognosis ranging from clinical improvement to being stable to clinical deterioration (rare <sup>5</sup>). Various treatment strategies have been described which include systemic and inhaled corticosteroids, bronchodilators, and lung resection.</p><h4>See also</h4><ul>- +</ul><h4>History and etymology</h4><p>The first clinical series of six cases describing DIPNECH was published by <strong> Aguayo </strong> et al in 1992.</p><h4>Treatment and prognosis</h4><p>The rarity of the condition poses some clinical challenge on establishing treatment and assessing prognosis. Limited studies suggest that the condition can have a variable prognosis ranging from clinical improvement to being stable to clinical deterioration (rare <sup>5</sup>). Various treatment strategies have been described which include systemic and inhaled corticosteroids, bronchodilators, and lung resection.</p><h4>See also</h4><ul>