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. 2019 Jun;98(26):e16086.
doi: 10.1097/MD.0000000000016086.

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

Elena Bargagli  1 , Maria Antonietta Mazzei  2 , Martina Orlandi  3 , Francesco Gentili  2 , Francesca Bellisai  4 , Bruno Frediani  4 , Laura Bergantini  1 , Loredana Carobene  1 , Silvia Bellando Randone  3 , Serena Guiducci  3 , Paolo Cameli  1 , Cosimo Bruni  3 , Marco Matucci Cerinic  3
Affiliations

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

Elena Bargagli et al. Medicine (Baltimore). 2019 Jun.

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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Conflict of interest statement

All these authors have no potential conflicts of interest.

Figures

Figure 1
Figure 1
(A–F) PPFE (A–C) in an 80-year-old woman with scleroderma involving the upper lobes and the apical segments of the inferior lobes including the fissures. Three years after the diagnosis (D–F) it is dear a mid increase of fibrosis, especially on the right side (arrows in B, C, E, F). The esophagus is dilated with food stagnation (arrowhead in D) due to the scleroderma involvement. PPFE = pleuroparenchymal fibroelastosis.
Figure 2
Figure 2
(A, B) PPFE in a 59-year-old woman with scleroderma and allergic asthma. Coronal-oblique (A) and sagittal (B) multiplanar reconstructions show the predominant involvement of the upper lobe and apical segment of the lower lobe on the right side. Note the involvement of the major fissure (arrow in B) that is atypical finding in PPFE. PPFE = pleuroparenchymal fibroelastosis.
Figure 3
Figure 3
FVC (%), FEV1 (%), and DLCO (%) predicted values in patients at the time of diagnosis and after 2 years of follow-up (∗P < .05). DLCO = diffuse capacity for carbon monoxide, FEV1 = forced expiratory volume in 1 second, FVC = forced vital capacity.
Figure 4
Figure 4
Bronchoalveolar lavage cellular populations from a patient affected by PPFE and systemic sclerosis. PPFE = pleuroparenchymal fibroelastosis.
See this image and copyright information in PMC

References

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