DIPNECH: An International Case Series

<bold>Aims and objectives:</bold> DIPNECH is a rare pulmonary disorder that is characterized by diffuse bronchial hyperplasia and bronchial neuroendocrine cells. The incidence and prevalence is unknown. Presenting symptoms may be non-specific and the clinical course varies, making diagnosis challenging. We present a multi-centre descriptive case series of DIPNECH. <bold>Methods:</bold> Data from 15 international centres was collated. Data collected included presentation, PFTs, histopathology, radiology and outcomes. <bold>Results:</bold> Of the 221 patients with DIPNECH; 92.31% were female (n=172), mean age 67.65 (±63.93)yr. 35.44% were ex-smokers. Cough (51.20%) and SOB (32%) were the most common symptoms; 19.29% were asymptomatic (n=38). 45.27% of patients (n=67) had an obstructive pattern on PFTs. Mean FEV1 was 68.65 (±24.12)% predicted. Diagnosis was biopsy-confirmed in 98.08% of cases. Typical carcinoid was diagnosed in 52.82%. Pulmonary nodules (99.53%) and mosaic attenuation (73.18%) were the most prevalent radiological findings. There was a significant association between the presence of nodules and symptoms (P<0.001), and nodules and obstructive PFTs (P<0.001). 26.59% of patients received no treatment. 16.77% (n= 69) of patients had symptomatic relief with bronchodilators, 13.64% (n= 65) with ICS inhalers. 41 patients were treated with SSA with symptomatic improvement reported in 12.36%. 8 patients received mTOR inhibitors. 2 patients within this cohort died from DIPNECH. <bold>Conclusions:</bold> Diagnosis and management of DIPNECH is challenging due to the rarity of the condition and sparse data. We believe this to be the largest descriptive case series to date and hope this data will build our understanding of this rare disease and guide future collaborative research and care.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.oa1060
Primary Topic
Tracheal and airway disorders
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article
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article

DIPNECH: An International Case Series

Camille Taillé, Bilal F. Samhouri, Rachel Crowley, Vincent Cottin et al.
Tracheal and airway disorders
article

DIPNECH: An International Case Series

Camille Taillé, Bilal F. Samhouri, Rachel Crowley, Vincent Cottin, Marcel Veltkamp, Rémi Diesler, Michael P. Keane, Lykourgos Kolilekas, Marissa O’Callaghan, Cormac McCarthy, Zoe Daniil, Francesco Bonella, Dermot O’Toole, Filippo Lococo, Aurélie Fabre, Aggeliki Lazaratou, Nishant Gupta, Sarah H. Forde, Alessandro N. Franciosi, Ilias Dimeas, Demosthenes Bouros, Jay Hoon Ryu, Jonathan Strosberg, Spyros A. Papiris, Adam J. Byrne, Effrosyni D. Manali, David J. Murphy, Sergio Harari, M. Penugonda, Francis X. McCormack, Hussein Almeamar, Helen O’Brien, Ludovic Fournel, Laurie Carr
article en

Abstract

<bold>Aims and objectives:</bold> DIPNECH is a rare pulmonary disorder that is characterized by diffuse bronchial hyperplasia and bronchial neuroendocrine cells. The incidence and prevalence is unknown. Presenting symptoms may be non-specific and the clinical course varies, making diagnosis challenging. We present a multi-centre descriptive case series of DIPNECH. <bold>Methods:</bold> Data from 15 international centres was collated. Data collected included presentation, PFTs, histopathology, radiology and outcomes. <bold>Results:</bold> Of the 221 patients with DIPNECH; 92.31% were female (n=172), mean age 67.65 (±63.93)yr. 35.44% were ex-smokers. Cough (51.20%) and SOB (32%) were the most common symptoms; 19.29% were asymptomatic (n=38). 45.27% of patients (n=67) had an obstructive pattern on PFTs. Mean FEV1 was 68.65 (±24.12)% predicted. Diagnosis was biopsy-confirmed in 98.08% of cases. Typical carcinoid was diagnosed in 52.82%. Pulmonary nodules (99.53%) and mosaic attenuation (73.18%) were the most prevalent radiological findings. There was a significant association between the presence of nodules and symptoms (P<0.001), and nodules and obstructive PFTs (P<0.001). 26.59% of patients received no treatment. 16.77% (n= 69) of patients had symptomatic relief with bronchodilators, 13.64% (n= 65) with ICS inhalers. 41 patients were treated with SSA with symptomatic improvement reported in 12.36%. 8 patients received mTOR inhibitors. 2 patients within this cohort died from DIPNECH. <bold>Conclusions:</bold> Diagnosis and management of DIPNECH is challenging due to the rarity of the condition and sparse data. We believe this to be the largest descriptive case series to date and hope this data will build our understanding of this rare disease and guide future collaborative research and care.

Université Claude Bernard Lyon 1 (FR), University College Dublin (IE), Università Cattolica del Sacro Cuore (IT), Mayo Clinic (US), National and Kapodistrian University of Athens (GR), Moffitt Cancer Center (US), National Jewish Health (US), Ruhrlandklinik (DE), Université Sorbonne Paris Nord (FR), Hôpital Cochin (FR), Assistance Publique – Hôpitaux de Paris (FR), St. Antonius Ziekenhuis (NL), University Hospital of Larissa (GR), St. Vincent's University Hospital (IE), Hôpitaux Universitaires Paris-Ouest (FR), Hôpital Louis Pradel (FR), Mayo Clinic in Florida (US), Ospedale San Giuseppe (IT), Oncology Hematology Care (US), Sotiria General Hospital (GR), Jewish Hospital (US), University of Colorado Denver (US)
Openalex Percentile: Top 63%
Tracheal and airway disorders
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