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Original Research: Diffuse Lung Disease |

The Clinical Course of Diffuse Idiopathic Pulmonary Neuroendocrine Cell HyperplasiaCourse of Pulmonary Neuroendocrine Hyperplasia

Laurie L. Carr, MD; Jonathan H. Chung, MD; Rosane Duarte Achcar, MD; Zoran Lesic, MD; Ji Y. Rho, MD; Kunihiro Yagihashi, MD; Robert M. Tate, MD; Jeffrey J. Swigris, DO; Jeffrey A. Kern, MD
Author and Funding Information

From the Division of Oncology (Drs Carr and Kern), Division of Radiology (Drs Chung and Yagihashi), Division of Pathology (Dr Duarte Achcar), and Division of Pulmonary, Critical Care and Sleep Medicine (Drs Tate and Swigris), Department of Medicine, National Jewish Health, Denver, CO; Department of Medicine (Dr Lesic), St. Anthony Hospital, Lakewood, CO; Division of Radiology (Dr Rho), CHA Bundang Medical Center, CHA University, Seoul, Korea; and Department of Radiology (Dr Yagihashi), St Marianna University School of Medicine, Kanagawa, Japan.

CORRESPONDENCE TO: Laurie L. Carr, MD, Division of Oncology, Department of Medicine, National Jewish Health, 1400 Jackson St, Denver, CO 80206; e-mail: carrl@njhealth.org


FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;147(2):415-422. doi:10.1378/chest.14-0711
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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.

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