title: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview.
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.
authors.affiliation: Division of Internal Medicine, Department of Medicine, Mayo Clinic, Scottsdale, AZ 85259-5499, USA.
authors.forename: Adrienne A
authors.initials: AA
authors.lastname: Nassar
authors.forename: Dawn E
authors.initials: DE
authors.lastname: Jaroszewski
authors.forename: Richard A
authors.initials: RA
authors.lastname: Helmers
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
authors.forename: Bhavesh M
authors.initials: BM
authors.lastname: Patel
authors.forename: Farouk
authors.initials: F
authors.lastname: Mookadam
mesh_terms: D003371:Cough / Q000209:etiology; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D055613:Multiple Pulmonary Nodules / Q000175:diagnosis / Q000473:pathology; D055099:Neuroendocrine Cells / Q000473:pathology*; D011230:Precancerous Conditions / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D012129:Respiratory Function Tests