Lung Pathology: Student/Resident Case Report Poster - Lung Pathology II |

An Under Recognized Cause of Progressive Dyspnea FREE TO VIEW

Avinash Ramdass, MD; Abubakr Bajwa, MD; Mariam Louis, MD; Kamalpreet Parmar, MD; Arun Gopinath, MD; Vandana Seeram, MD
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Department of Pulmonary and Critical Care, University of Florida Jacksonville, Jacksonville, FL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):816A. doi:10.1016/j.chest.2016.08.912
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SESSION TITLE: Student/Resident Case Report Poster - Lung Pathology II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disease with insidious onset that is being recognized with increasing frequency as a cause of progressive dyspnea.

CASE PRESENTATION: A 67-year-old female non-smoker, was referred for progressive dyspnea on exertion and dry cough that failed to respond to empiric asthma therapy. She had no occupational or environmental exposures. An HRCT chest performed at symptom onset showed perihilar bronchiectasis and mild interstitial fibrosis predominantly in the lower lobes with mosaicism. Cardiac and rheumatologic workup was unremarkable. PFTs showed a mild restrictive pattern with a normal diffusing capacity but air trapping was noted. Bronchoscopy was unremarkable with negative BAL cytology and cultures. Progressive hypoxia prompted a repeat HRCT which showed worsening diffuse mosaicism in the upper and lower lobes (Figure 1). A surgical lung biopsy showed multiple neuroendocrine tumorlets that were positive for synaptophysin and chromogranin on a background of acute and chronic inflammation with fibroblastic proliferation and mild interstitial fibrosis (Figure 2).

DISCUSSION: The majority of patients diagnosed with DIPNECH are middle-aged nonsmoking females, with nonspecific respiratory symptoms, commonly misdiagnosed as asthma or chronic obstructive pulmonary disease. The gold standard for diagnosis remains surgical lung biopsy with demonstration of neuroendocrine cell proliferation confined to the epithelium of large and small airways. Tumorlets can be localized or diffuse and commonly produce bombesin and gastrin-releasing peptides that stimulate fibroblasts leading to peribronchiolar and interstitial fibrosis. PFTs show obstruction but may be restrictive when there is progression of fibrosis. Common HRCT findings are ground-glass opacities, mosaicism, endobronchial wall thickening and nodules. Reported 5 year survival is 83%. Treatment options include observation or trial of inhaled or systemic corticosteroids. Octreotide/somatostatin analogs have been suggested but evidence is lacking. Patients with severe respiratory symptoms should be evaluated for lung transplantation.

CONCLUSIONS: The diagnosis of DIPNECH is often delayed due to its insidious onset and nonspecific symptoms. PFTs may be obstructive or restrictive depending on the degree of underlying fibrosis. The gold standard for diagnosis remains surgical lung biopsy and there is currently no consensus for treatment.

Reference #1: Nassar AA. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: a systematic overview. Am J Respir Crit Care Med. 2011 Jul 1; 184(1):8-16.

Reference #2: Davies SJ. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognized spectrum of disease. Thorax 2007; 62:248-252.

Reference #3: Wirtschafter E. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia of the Lung (DIPNECH): Current Best Evidence. Lung. 2015 Oct;193(5):659-67.

DISCLOSURE: The following authors have nothing to disclose: Avinash Ramdass, Abubakr Bajwa, Mariam Louis, Kamalpreet Parmar, Arun Gopinath, Vandana Seeram

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