Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.
Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do? / Bargagli, E.; Mazzei, M. A.; Orlandi, M.; Gentili, F.; Bellisai, F.; Frediani, B.; Bergantini, L.; Carobene, L.; Bellando-Randone, S.; Guiducci, S.; Cameli, P.; Bruni, C.; Cerinic, M. M.. - In: MEDICINE. - ISSN 1536-5964. - 98:26(2019), pp. e16086-e16086. [10.1097/MD.0000000000016086]
Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do?
Orlandi M.;Guiducci S.;
2019
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.
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