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• W2980636091 abstract "SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: The World Health Organization(WHO) classification of lung tumors defines diffuse idiopathic pulmonary neuroendocrine cell hyperplasia(DIPNECH) as a preinvasive lesion of pulmonary carcinoids characterized by a proliferation of neuroendocrine cells confined to the mucosa of bronchi or forming tumorlets and carcinoids. Here, we present a case of DIPNECH. CASE PRESENTATION: 72-year-old female, former smoker, with PMHx of morbid obesity, OSA on CPAP and chronic cough (on albuterol) and was sent for abnormal images. She recently traveled to Aruba and noticed increased dyspnea on exertion. She had a CXR done which showed “water in her lungs” and was referred to pulmonology. She reported dry cough and progressive dyspnea on exertion for the last year, and all other ROS were negative. She was saturating 100% on RA and physical exam was unremarkable. CT chest showed multiple subpleural and bilateral upper lobe predominant nodules with ground-glass-opacities of unclear etiology. All labs including rheumatological were WNL. PFT suggested moderate restriction. Bronchoscopy showed no endobronchial lesions but transbronchial biopsy revealed small airway neuroendorcrine cell proliferation and no granulomas or malignant cells consistent with DIPNECH. DISCUSSION: DIPNECH was first defined in 1992 by Aguayo et al. and as studies have continued exploring the disease, there has been some discussion into what classified as DIPNECH. It has been used to describe two types of patients, one is based on their clinical, radiological, and pathological presentation, and the other is defined purely histologically in asymptomatic patients. Symptomatic DIPNECH has been diagnosed mostly in female non-smokers who present with cough, exertional dyspnea and wheezing. Due to their similar presentation, patients may be misdiagnosed with asthma or GERD. Abnormal LFTs is a typical lab finding. CT Images show air trapping or mosaicism. Diagnosis of DIPNECH requires histological confirmation of diffuse neuroendocrine cell proliferation and the presence of constrictive bronchiolitis by transbronchial biopsy, which is found in both types of DIPNECH described in literature. The course of DIPNECH is typically indolent but there are cases that report slow progression of DIPNECH leading to respiratory failure. Octreotide has been used for symptomatic coughing, but reports have not shown that it improves the course of the disease. Symptomatic treatment for obstruction and lung transplantation in select patients are the current therapeutic options for DIPNECH. CONCLUSIONS: DIPNECH is a rare disease and clinicians should have a high index of suspicion for patients presenting with indolent pulmonary symptoms. Reference #1: Davies, Susan J., et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax 62.3 (2007): 248-252. Reference #2: Aguayo, Samuel M., et al. Idiopathic diffuse hyperplasia of pulmonary neuroendocrine cells and airways disease. New England Journal of Medicine 327.18 (1992): 1285-1288. Reference #3: Wirtschafter, Eric, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia of the lung (DIPNECH): current best evidence. Lung 193.5 (2015): 659-667. DISCLOSURES: No relevant relationships by Mona Alipour, source=Web Response No relevant relationships by Khoi Paul Dang-Ho, source=Web Response No relevant relationships by Louis Gerolemou, source=Web Response No relevant relationships by Nabil Mesiha, source=Web Response" @default.
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• W2980636091 date "2019-10-01" @default.
• W2980636091 modified "2023-09-25" @default.
• W2980636091 title "A CASE REPORT OF DIFFUSE IDIOPATHIC PULMONARY NEUROENDOCRINE CELL HYPERPLASIA (DIPNECH)" @default.
• W2980636091 doi "https://doi.org/10.1016/j.chest.2019.08.1587" @default.
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