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Upper Lobe Predominant Fibrosing Interstitial Lung Diseases (ILDs): Prevalence, Causes, Characteristics and Prognosis
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A1688 - Upper Lobe Predominant Fibrosing Interstitial Lung Diseases (ILDs): Prevalence, Causes, Characteristics and Prognosis
Author Block: H. Nunes1, L. Mourtada1, L. Sese1, P. Brillet2, D. Bouvry1, M. Kambouchner3, D. Valeyre1; 1Service de Pneumologie, Centre de Référence des Maladies Pulmonaires Rares de l’Adulte, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France, 2Service de Radiologie, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France, 3Service d'Anatomie Pathologique, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France.
Introduction: Although the causes of upper lobe predominant fibrosing ILDs are known to be various, no systematic study is available in the literature. The aim of the study was (i) to determine the prevalence of upper lobe predominant fibrosing ILDs, and the distribution of underlying causes, and (ii) to describe their characteristics and prognosis according to underlying causes.
Methods: This is a retrospective study based on a cohort of 1132 patients with chronic fibrosing ILDs referred to our expert centre between 2006 and 2016. Patients with a definite diagnosis of sarcoidosis established before their referral were excluded.
Results: 87 patients had a predominance of fibrosis in upper lobes at chest high-resolution computed tomography (HRCT), representing a prevalence of 7.69%. After multidisciplinary discussion, the range of diagnoses was wide, including sarcoidosis (n=21), pleuroparenchymal fibroelastosis (PPFE : n=19), hypersensitivity pneumonitis (HP: n=15), unclassifiable ILD (n=11), smoking-related ILD (n=6), silicosis (n=5) and other (n=10). The 4 most prevalent groups were compared (sarcoidosis, PPFE, HP, and unclassifiable ILD). Patients with PPFE had a more frequent familial history of ILD (7/19, 37%), a lower BMI, more frequent occurrence of pneumothorax/pneumomediastinum (8/19, 42%) and no uptake on 18FDG-PET. At HRCT, sarcoidosis was more likely to show fibrotic masses, lymph nodes, and central predominance. Loss of volume, asymmetric lesions, sub-pleural consolidations were more frequent, and thorax was flatter in PPFE. Mosaic attenuation indicated HP. The annual decline of forced vital capacity was lesser in sarcoidosis. PPFE survival was significantly poorer as compared to other groups (p=0,02), estimated at 60% at 5 years, in particular in the presence of associated ILD in the bases.
Conclusion: Several clinical and radiological characteristics help distinguish the main causes of upper lobe predominant fibrosing ILDs. The high frequency of familial forms should incite genetic screening in PPFE, which prognosis is poor.
Author Block: H. Nunes1, L. Mourtada1, L. Sese1, P. Brillet2, D. Bouvry1, M. Kambouchner3, D. Valeyre1; 1Service de Pneumologie, Centre de Référence des Maladies Pulmonaires Rares de l’Adulte, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France, 2Service de Radiologie, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France, 3Service d'Anatomie Pathologique, AP-HP, Hôpital Avicenne, EA2363, Université Paris 13, Bobigny, France.
Introduction: Although the causes of upper lobe predominant fibrosing ILDs are known to be various, no systematic study is available in the literature. The aim of the study was (i) to determine the prevalence of upper lobe predominant fibrosing ILDs, and the distribution of underlying causes, and (ii) to describe their characteristics and prognosis according to underlying causes.
Methods: This is a retrospective study based on a cohort of 1132 patients with chronic fibrosing ILDs referred to our expert centre between 2006 and 2016. Patients with a definite diagnosis of sarcoidosis established before their referral were excluded.
Results: 87 patients had a predominance of fibrosis in upper lobes at chest high-resolution computed tomography (HRCT), representing a prevalence of 7.69%. After multidisciplinary discussion, the range of diagnoses was wide, including sarcoidosis (n=21), pleuroparenchymal fibroelastosis (PPFE : n=19), hypersensitivity pneumonitis (HP: n=15), unclassifiable ILD (n=11), smoking-related ILD (n=6), silicosis (n=5) and other (n=10). The 4 most prevalent groups were compared (sarcoidosis, PPFE, HP, and unclassifiable ILD). Patients with PPFE had a more frequent familial history of ILD (7/19, 37%), a lower BMI, more frequent occurrence of pneumothorax/pneumomediastinum (8/19, 42%) and no uptake on 18FDG-PET. At HRCT, sarcoidosis was more likely to show fibrotic masses, lymph nodes, and central predominance. Loss of volume, asymmetric lesions, sub-pleural consolidations were more frequent, and thorax was flatter in PPFE. Mosaic attenuation indicated HP. The annual decline of forced vital capacity was lesser in sarcoidosis. PPFE survival was significantly poorer as compared to other groups (p=0,02), estimated at 60% at 5 years, in particular in the presence of associated ILD in the bases.
Conclusion: Several clinical and radiological characteristics help distinguish the main causes of upper lobe predominant fibrosing ILDs. The high frequency of familial forms should incite genetic screening in PPFE, which prognosis is poor.
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