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Constrictive bronchiolitis in diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Bilal F Samhouri1, Natalya Azadeh2, Thorvardur R Halfdanarson3
1Dept of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
ERJ Open Research
|December 2, 2020
Summary
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) often affects women and can cause airflow obstruction. Current treatments show limited effectiveness for this condition.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a condition involving neuroendocrine cell proliferation within the lungs.
- It is part of the spectrum of pulmonary neuroendocrine tumors.
- The link between DIPNECH and airflow obstruction is not well understood.
Purpose of the Study:
- To investigate the clinical characteristics, imaging findings, and treatment outcomes of patients with DIPNECH.
- To clarify the association between DIPNECH and airflow obstruction.
Main Methods:
- A retrospective review of electronic medical records at Mayo Clinic.
- Inclusion criteria: biopsy-proven DIPNECH.
- Data extracted: clinical, pulmonary function, imaging, histopathology, treatments, and outcomes.
Main Results:
- The study included 44 patients, predominantly female (91%), with a median age of 65 years; 73% were never-smokers.
- Respiratory symptoms were common (86%), with 45% previously diagnosed with asthma or COPD.
- Pulmonary function tests revealed obstructive patterns in 52% of patients. Chest CT scans showed diffuse nodules (95%) and mosaic attenuation (77%).
- Treatment varied, with inhaled bronchodilators (55%) and corticosteroids (41%) being common. Octreotide was used in 32%.
- Of 24 patients with follow-up PFTs, 50% remained stable, 33% worsened, and 17% improved over a median of 21.3 months.
Conclusions:
- DIPNECH primarily affects women and is characterized by diffuse pulmonary nodules and mosaic attenuation on imaging.
- Airflow obstruction, likely due to constrictive bronchiolitis, is a common complication.
- Current pharmacological therapies demonstrate limited efficacy in managing DIPNECH-associated airflow obstruction.
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