Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

Abstract

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is being recognized with increasing frequency. Diagnostic and treatment information is limited. A systematic review is presented, focusing on patient demographics, clinical presentation, diagnosis, treatment options, and outcomes. A systematic electronic literature search was conducted for adult DIPNECH cases reported in the English literature during the past 6 years. Twenty-four DIPNECH cases were identified. Another case from our institution is contributed. Women represent 92% (23 of 25). Mean age at diagnosis was 58 years (range, 36-76 yr). Most were nonsmokers (16 of 24). Symptoms included cough (71%), dyspnea (63%), and wheezing (25%) occurring days to years before diagnosis. Pulmonary function testing showed obstructive ventilatory disease in 54%. Lung nodules were seen in 15 patients (63%), ground-glass attenuation in 7 patients (29%), and bronchiectasis in 5 patients (21%). Histological confirmation required surgical lung biopsy for 88%; however, transbronchial biopsies alone were diagnostic in three patients. Treatments strategies included systemic and inhaled corticosteroids, bronchodilators, and lung resection. Available follow-up data in 17 patients showed 6 clinically improved, 7 who remained stable, and 4 clinically deteriorated. The majority of patients presenting with DIPNECH are middle-aged females with symptoms of cough and dyspnea; obstructive abnormalities on pulmonary function testing; and radiographic imaging showing pulmonary nodules, ground-glass attenuation, and bronchiectasis. In general, the clinical course remains stable; however, progression to respiratory failure does occur. Long-term follow-up and treatment remains incomplete. Establishment of a national multicenter DIPNECH registry would allow formulation of optimal evidence-based guidelines for management of these patients.

Pulmonary Neuroendocrine Cell Hyperplasia Associated With Surfactant Protein C Gene Mutation

“…His CT of chest finding revealed emphysema, ground-glass opacities, multiple lung cysts, septal thickening, and subpleural honeycombing, which have all been reported to be associated with SFTPC mutations. In addition, there was some evidence of air-trapping and mosaic attenuation, which are well-described radiographic findings in DIPNECH [23,33].…”

A Case of DIPNECH Presenting as Usual Interstitial Pneumonia

“…The presence of diffuse proliferation of PNEC without the aforementioned risk factors is suggestive of DIPNECH. It is more prevalent in females (92%) with a mean age at presentation of 58 years [2]. Based on a review of literature by Nassar et al, the most common presenting symptoms reported are cough (71%), dyspnea (63%), and wheezing (25%) [2].…”

“…It is more prevalent in females (92%) with a mean age at presentation of 58 years [2]. Based on a review of literature by Nassar et al, the most common presenting symptoms reported are cough (71%), dyspnea (63%), and wheezing (25%) [2]. The most common finding www.pneumonologia.viamedica.pl on PFTs were an irreversible obstructive defect (54−78% of patients), however, mixed (17%), and restrictive pattern (13%) were also noted [2].…”

“…Based on a review of literature by Nassar et al, the most common presenting symptoms reported are cough (71%), dyspnea (63%), and wheezing (25%) [2]. The most common finding www.pneumonologia.viamedica.pl on PFTs were an irreversible obstructive defect (54−78% of patients), however, mixed (17%), and restrictive pattern (13%) were also noted [2]. The presence of different types of PFT patterns can be explained by the underlying pathophysiology.…”

“…Single or multiple pulmonary nodules were the most common CT finding in patients with DIPNECH [2]. Mosaic perfusion is also a very commonly reported CT finding in DIPNECH that results from vasoconstriction in the areas of small airway obstruction [3].…”