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Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience

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Abstract

Background

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5 mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease.

Methods

A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology.

Results

Our patient cohort consisted of twelve females and one male with a mean age of 63 years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of long-acting somatostatin analog and everolimus. Five patients had concomitant bronchial carcinoids.

Conclusions

DIPNECH is a rare pathology that can profoundly affect a patient’s quality of life. Paroxysmal coughing episodes can be difficult to treat. Our limited single center experience shows encouraging response to use of somatostatin analogs, azithromycin, and everolimus in the management of debilitating DIPNECH associated symptoms.

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Acknowledgements

The authors thank Catherine Anthony, Ph.D. for assistance with manuscript preparation.

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Authors and Affiliations

  1. Division of Medical Oncology, Department of Internal Medicine, University of Kentucky, Lexington, KY, USA

    Zin W. Myint, Aman Chauhan & Lowell B. Anthony

  2. Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Kentucky, Lexington, KY, USA

    James McCormick

  3. College of Medicine, University of Kentucky, Lexington, KY, USA

    Elizabeth Behrens

Authors
  1. Zin W. Myint
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  2. James McCormick
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  3. Aman Chauhan
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  4. Elizabeth Behrens
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  5. Lowell B. Anthony
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Contributions

Conception and design: All authors; Administrative support: LA; Provision of materials: All authors; Collection and assembly of data: ZM and AC; Data interpretation: ZM; Manuscript writing and final approval: All authors.

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Correspondence to Zin W. Myint.

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Myint, Z.W., McCormick, J., Chauhan, A. et al. Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience. Lung 196, 577–581 (2018). https://doi.org/10.1007/s00408-018-0149-z

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  • DOI: https://doi.org/10.1007/s00408-018-0149-z

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