Chest
Postgraduate Education CornerPulmonary and Critical Care PearlsA 66-Year-Old Woman With Dry Cough and Exertional Dyspnea
Section snippets
Physical Examination
The patient's vital signs at rest were as follows: heart rate, 78/min (regular); respiratory rate, 16 breaths/min; oxygen saturation, 97% on room air; temperature, 36.6°C. Her BMI was 29. Chest examination revealed bilateral expiratory wheezes. The patient's oxygen saturation dropped to 90% after walking for 200 m on flat ground. The remainder of the physical examination results were normal.
Laboratory Findings
Hemogram, blood chemistry, and urine studies on admission were within normal limits. Spirometry demonstrated an obstructive ventilatory defect characterized by an FEV1 of 1.08 L (48% predicted), an FVC of 1.70 (63.9% predicted), and an FEV1/FVC of 63%. The bronchodilation test result was negative. Chest radiograph showed a mild peribronchial wall thickening at the lung bases. High-resolution CT (HRCT) scan revealed the presence of areas of variable lung attenuation (mosaic pattern) along with
What is the diagnosis?
Diagnosis: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Discussion
The term diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) refers to diffuse collections of scattered single cells, small nodules, or linear proliferation of neuroendocrine cells, which are confined to the airway mucosa. When the proliferation of neuroendocrine cells extends beyond the basement membrane it is referred to as tumorlet, and if it forms nodules >5 mm it is termed carcinoid tumor. The whole spectrum of neuroendocrine lesions described here is not infrequently seen in the
Clinical Pearls
- 1.
DIPNECH is a pulmonary disorder characterized by diffuse proliferation of neuroendocrine cells into the small airways. It is often associated, in the same patient, with more invasive neuroendocrine lesions, such as tumorlets and carcinoid tumors, of which it is considered a preneoplastic state.
- 2.
In a considerable percentage of cases, DIPNECH is associated with constrictive bronchiolitis, which is probably triggered by neuropeptides secreted by the neuroendocrine cells and is believed to be the
Acknowledgments
Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Other contributions: The work was performed at Maggiore Hospital, Bologna, Italy.
References (0)
Cited by (3)
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: DIPNECH
2021, Current Opinion in Pulmonary MedicineDiffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome
2016, European Respiratory Journal
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.