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. - ELETTRONICO. - 98:(2019), pp. e16086-e16086. [10.1097/MD.0000000000016086]

Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do?

Orlandi M.;Frediani B.;Carobene L.;Bellando-Randone S.;Guiducci S.;Cameli P.;Bruni C.;Cerinic M. M.
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.
2019
98
e16086
e16086
Bargagli E.; Mazzei M.A.; Orlandi M.; Gentili F.; Bellisai F.; Frediani B.; Bergantini L.; Carobene L.; Bellando-Randone S.; Guiducci S.; Cameli P.; B...espandi
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1192899
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