Merel E Hellemons1,2,3, Catharina C Moor1,2,3, Jan von der Thüsen4,2, Mariska Rossius5,2, Arlette Odink5,2, Laila Haugen Thorgersen6, Johny Verschakelen7,2, Wim Wuyts8,2,9, Marlies S Wijsenbeek10,2,9, Elisabeth Bendstrup11,2,9. 1. Dept of Interstitial Lung Disease, Erasmus Medical Center, Rotterdam, The Netherlands. 2. This centre is a member of the European Reference Network for Rare Lung Diseases/Interstitial Lung Diseases (ERN-LUNG ILD). 3. These authors share first authorship. 4. Dept of Pathology, Erasmus Medical Center, Rotterdam, The Netherlands. 5. Dept of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands. 6. Dept of Respiratory Diseases, Nastved Hospital, Nastved, Denmark. 7. Dept of Radiology, University Hospital Leuven, Leuven, Belgium. 8. Unit of Interstitial Lung Disease, University Hospital Leuven, Leuven, Belgium. 9. These authors share senior authorship. 10. Dept of Interstitial Lung Disease, Erasmus Medical Center, Rotterdam, The Netherlands m.wijsenbeek-lourens@erasmusmc.nl. 11. Dept of Respiratory Diseases and Allergy, Center for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark.
Abstract
BACKGROUND: Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. METHODS: A systematic literature search was performed for all original cases of adult patients with histopathologically-confirmed DIP. Individual patient data were extracted and summarised. RESULTS: We included 68 individual cases and 13 case series reporting on 294 cases. Most common presenting symptoms were dyspnoea and cough. Pulmonary function showed a restrictive pattern (71%) with decreased diffusion capacity. We found a high incidence (81%) of ever smoking in patients with DIP and 22% of patients had other (occupational) exposures. Characteristic features on high-resolution computed tomography (HRCT) scan were bilateral ground-glass opacities with lower lobe predominance (92%). Treatment and duration of treatment widely varied. Initial response to treatment was generally good, but definitely not uniformly so. A significant proportion of patients died (25% of individual cases) or experienced a relapse (18% of individual cases). CONCLUSION: DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology.
BACKGROUND: Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. METHODS: A systematic literature search was performed for all original cases of adult patients with histopathologically-confirmed DIP. Individual patient data were extracted and summarised. RESULTS: We included 68 individual cases and 13 case series reporting on 294 cases. Most common presenting symptoms were dyspnoea and cough. Pulmonary function showed a restrictive pattern (71%) with decreased diffusion capacity. We found a high incidence (81%) of ever smoking in patients with DIP and 22% of patients had other (occupational) exposures. Characteristic features on high-resolution computed tomography (HRCT) scan were bilateral ground-glass opacities with lower lobe predominance (92%). Treatment and duration of treatment widely varied. Initial response to treatment was generally good, but definitely not uniformly so. A significant proportion of patientsdied (25% of individual cases) or experienced a relapse (18% of individual cases). CONCLUSION: DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology.
Authors: Simona Gurzu; Catalin Bogdan Satala; Lorena Elena Melit; Adrian Streinu-Cercel; Dan Otelea; Brandusa Capalna; Claudiu Ioan Puiac; Janos Szederjesi; Ioan Jung Journal: Front Pediatr Date: 2020-11-11 Impact factor: 3.418