Chest
Original Research: Diffuse Lung DiseaseThe Clinical Course of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia
Section snippets
Subjects
This study was conducted in accordance with the amended Declaration of Helsinki. This protocol was approved by the National Jewish Health (NJH) Institutional Review Board (HS#2530). Ninety-three potential subjects were identified by query of the NJH Clinical Research Database for “neuroendocrine hyperplasia” and “age ≥ 18” and “high-resolution chest CT.” Of these 93 patients, 21 could be verified as having DIPNECH by a pathology report. From June 2011 to May 2013, nine additional patients were
Results
All subjects were women. The average age at diagnosis was 62 years. Baseline characteristics are presented in Table 1. The majority of subjects (63%) never smoked, and the remaining subjects had quit smoking prior to DIPECH diagnosis. The most common symptoms were chronic cough and dyspnea. In general, cough developed first, often more than 10 years prior to diagnosis. Medical records for the time prior to receiving a DIPNECH diagnosis were available for 27 subjects. Of these, 12 had been given
Discussion
We identified 30 patients with DIPNECH followed at our institution and detailed their clinical characteristics and disease course over time. To our knowledge, this DIPNECH cohort is the largest assembled. We observed a disease that predominately affects middle-aged women with slowly progressive physiologic lung obstruction. The striking sex bias of this disease has been observed in previous reports and remains unexplained.5
We observed substantial heterogeneity in longitudinal disease behavior.
Conclusions
DIPNECH is a neuroendocrine cell proliferation that is predominately seen in middle-aged women. The diagnosis often is delayed until moderate to severe obstruction is present. This disease needs to be considered in the setting of obstructive physiology, particularly when diffuse pulmonary nodules and mosaic pattern air trapping are present on HRCT scan. Further studies are needed to improve understanding of the pathogenesis of this disease, particularly the sex bias, and to test the proposed
Acknowledgments
Author contributions: L. L. C. had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. L. L. C., J. H. C., R. D. A., and Z. L. contributed to the study design, data analysis, interpretation of results, writing and revision of the manuscript, and final approval of the manuscript; J. Y. R. and K. Y. contributed to the data collection and revision and final approval of the manuscript; R. M. T. and J. A. K.
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FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.