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Updated: Jul 14, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Neuroendocrine cell hyperplasia as an unusual form of interstitial lung disease
1Servei de Pneumologia, Hospital Universitari Vall d'Hebron, Departement de medicina UAB, Passeig Vall d'Hebron, Barcelona, Spain.
Insights
Pulmonary neuroendocrine cell hyperplasia (PNECH) can cause interstitial lung disease (ILD) that mimics other conditions. Early diagnosis and treatment led to a favorable outcome in these two reported cases.
Area of Science:
- Pulmonology
- Pathology
- Cell Biology
Background:
- Pulmonary neuroendocrine cell hyperplasia (PNECH) is a rare cause of interstitial lung disease (ILD).
- Few cases of PNECH presenting as ILD have been reported globally.
- Diagnosis requires open lung biopsy with specific neuroendocrine cell stains.
Observation:
- Two patients with ILD secondary to PNECH were identified among 500 ILD cases.
- Clinical presentation included cough, dyspnea, and restrictive respiratory function.
- Radiological findings showed mosaic ground-glass infiltrates, resembling non-specific interstitial pneumonia.
Findings:
- PNECH was the sole cause of diffuse lung disease in both patients.
- Biopsies confirmed PNECH, and patients received treatment.
- Both patients experienced a favorable outcome following diagnosis and intervention.
Implications:
- PNECH-related ILD can be misdiagnosed as idiopathic interstitial pneumonias.
- Understanding PNECH's role is crucial for accurate ILD diagnosis.
- Further research into the etiopathogenic role of neuroendocrine cells in lung fibrosis is warranted.
Study Objectives:
Two patients diagnosed with interstitial lung disease (ILD) secondary to pulmonary neuroendocrine cell (PNEC) hyperplasia are presented.
Background:
Pulmonary neuroendocrine cell hyperplasia (PNECH) is a rare entity worldwide and few cases presenting as ILD have been reported. Following the consensus criteria established by the American Thoracic Society (ATS) and the European Respiratory Society (ERS), three patients from among 500 ILD patients over the last 10 years in our institution were diagnosed with PNECH. The diagnosis was established by open lung biopsy using specific stains to demonstrate neuroendocrine cells in lung tissue.
Methods:
Patients were questioned on their medical and pathological history, occupational or environmental exposure to toxic substances and any relationship with smoking. In addition, were recorded symptoms at presentation, physical signs, analytic and respiratory function results, chest X-ray and CT scan features, bronchoscopy findings including bronchoalveolar lavage and transbronchial biopsy, and open lung biopsy findings.
Results:
In these two patients, PNECH was the only cause of their diffuse lung disease. Clinical signs and symptoms (cough, expectoration and progressive dyspnea) were similar to other idiopathic interstitial pneumonias and radiologic features (ground-glass infiltrates in mosaic pattern) were consistent with those of non-specific interstitial pneumonia or the onset of hypersensitivity pneumonitis. Functional respiratory alterations were consistent with restrictive pattern. Transbronchial and open biopsy findings are described, as well as the treatment and course of the disease. The two patients had a favorable outcome.
Conclusions:
Two cases of ILD secondary to PNEC hyperplasia are presented, with clinical and radiological findings that might be mistaken for other types of idiopathic interstitial pneumonias. The disease course is described and the possible etiopathogenic role that PNECs might play in the development of lung fibrosis is discussed.
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