Clinical characteristics of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
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O'Callaghan Marissa, Forde Sarah H, Franciosi Alessandro N, Penugonda Maitreyi, Diesler Remi, O'Brien Helen, Buikhuisen Wieneke, Almeamar Hussein, Samhouri Bilal F, Ryu Jay H, Veltkamp Marcel, Manali Effrosyni D, Lazaratou Aggeliki, Papiris Spyros A, Bonella Francesco, Carr Laurie, Cottin Vincent, Taille Camille, McCormack Francis X, Gupta Nishant, Strosberg Jonathan, Lococo Fillipo M, Harari Sergio, Pelosi Giuseppe, Papa Riccardo, Bouros Demosthenes, Kolilekas Lykourgos, Daniil Zoe, Dimeas Ilias, Hernandez-Gonzalez Fernanda, Sellares Jacobo, Spagnolo Paolo, Crowley Rachel K, O' Toole Dermot, O'Shea Donal, Quinn Sean, Murphy David J, Fabre Aurelie, Byrne Adam J, Keane Michael P, Fournel Ludovic, McCarthy Cormac
Аннотация
METHODS: Data were collated from 18 international centres. Information collected included disease presentation, pulmonary function testing, histopathology, radiological patterns and outcomes. The relationship between clinical features, radiology and symptoms were explored in parametric and nonparametric group-wise analyses, univariate linear regressions, and multivariate binomial logistic regression.
RESULTS: The mean±sd age of the 258 patients in this study was 63.3±10.6 years and 93.4% were female. Diffuse pulmonary nodules (98.8%) and mosaic attenuation (59.1%) were the most common radiological findings and 29.5% had obstructive spirometry with a mean±sdforced expiratory volume in 1 s (FEV1) % pred of 69.0±23.7%. There was a significant association between the number of nodules and a reduction in FEV1 % pred (p<0.001), while the presence of bronchial wall thickening on imaging was most closely associated with cough (OR 4.97, p=0.001) dyspnoea (OR 3.14, p=0.003) and bronchodilator responsiveness (OR 3.09, p=0.013). Approximately half of patients treated with inhaled beta agonist and corticosteroids (46.3%) or somatostatin analogue (54.1%) reported improvement in symptoms.
CONCLUSIONS: The presence of radiological bronchial wall thickening is associated with the presence of symptoms, while mosaic attenuation is correlated with airflow obstruction; hence, the presence of these radiological findings has the potential to guide possible treatment decisions.