title: The clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
abstract: BACKGROUND
  Current understanding of the clinical course of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is poor and based predominantly on small case series. In our clinical experience, we have found that the diagnosis of DIPNECH is frequently delayed because respiratory symptoms are ascribed to other lung conditions. The objectives of this study were to collect and analyze longitudinal clinical data on pulmonary physiology, chest high-resolution CT (HRCT) imaging, and therapies to better delineate the course of disease.
  
  
  METHODS
  We established a cohort of patients (N = 30) with DIPNECH seen at our institution. We used descriptive statistics to summarize cohort characteristics and longitudinal analytic techniques to model FEV1 % predicted (FEV1%) over time.
  
  
  RESULTS
  All subjects were women who presented with long-standing cough and dyspnea. The majority had an FEV1% < 50% at the time of diagnosis. Forty percent were given a diagnosis of asthma as the cause for physiologic obstruction. The mean FEV1% for the entire cohort showed no statistically significant decline over time, but 26% of the subjects experienced a 10% decline in FEV1 within 2 years. Among the pathology samples available for review, 28% (five of 18) had typical carcinoids and 44% had associated constrictive bronchiolitis. We propose clinical diagnostic criteria for DIPNECH that incorporate demographic, pulmonary physiology, HRCT imaging, and transbronchial and surgical lung biopsy data.
  
  
  CONCLUSIONS
  DIPNECH is a female-predominant lung disease manifested by dyspnea and cough, physiologic obstruction, and nodules on HRCT imaging. Additional research is needed to understand the natural history of this disease and validate the proposed diagnostic criteria.
authors.affiliation: Division of Oncology, Denver, CO. Electronic address: carrl@njhealth.org.
authors.forename: Laurie L
authors.initials: LL
authors.lastname: Carr
authors.affiliation: Division of Radiology, Denver, CO.
authors.forename: Jonathan H
authors.initials: JH
authors.lastname: Chung
authors.affiliation: Division of Pathology, Denver, CO.
authors.forename: Rosane
authors.initials: R
authors.lastname: Duarte Achcar
authors.affiliation: Department of Medicine, National Jewish Health, Denver, CO.
authors.forename: Zoran
authors.initials: Z
authors.lastname: Lesic
authors.affiliation: Department of Medicine, St. Anthony Hospital, Lakewood, CO.
authors.forename: Ji Y
authors.initials: JY
authors.lastname: Rho
authors.affiliation: Division of Radiology, Denver, CO; Division of Radiology, CHA Bundang Medical Center, CHA University, Seoul, Korea.
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yagihashi
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, Denver, CO.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Tate
authors.affiliation: Department of Radiology, St Marianna University School of Medicine, Kanagawa, Japan.
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Swigris
authors.affiliation: Division of Oncology, Denver, CO.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Kern
mesh_terms: D001988:Bronchiolitis / Q000473:pathology; D049109:Cell Proliferation; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D006965:Hyperplasia / Q000473:pathology; D008168:Lung / Q000166:cytology* / Q000000981:diagnostic imaging; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D055613:Multiple Pulmonary Nodules / Q000150:complications; D055099:Neuroendocrine Cells / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed