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Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias.

Portillo K, Guasch I, Becker C, Andreo F, Fernández-Figueras MT, Ramirez Ruz J, Martinez-Barenys C, García-Reina S, Lopez de Castro P, Sansano I, Villar A, Ruiz-Manzano J - Case Rep Pulmonol (2015)

Bottom Line: The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections.That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team.We present the first reported cases in Spain.

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.

ABSTRACT
Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.

No MeSH data available.


Related in: MedlinePlus

Case  2. (a) Axial HRCT scan shows very discrete lineal opacities in both lung apices. (b) Axial HRCT scan at the same level compared to (a) two years and two months later shows a loss of volume and prominent parenchymal fibrotic infiltrates. (c) Axial HRCT image at the level of carina reveals lung architectural distortion, as well as the presence of pleural thickening and subpleural parenchymal opacities.
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fig7: Case  2. (a) Axial HRCT scan shows very discrete lineal opacities in both lung apices. (b) Axial HRCT scan at the same level compared to (a) two years and two months later shows a loss of volume and prominent parenchymal fibrotic infiltrates. (c) Axial HRCT image at the level of carina reveals lung architectural distortion, as well as the presence of pleural thickening and subpleural parenchymal opacities.

Mentions: However, the second HRCT, performed during the period in which the patient experienced the spontaneous pneumothorax, revealed that the upper linear opacities had progressed to pulmonary infiltrates of fibrotic appearance. Also evident was a thickening of the visceral pleura, clearly visible due to the adjacent pneumothorax (Figure 6). Finally, in a HRCT made 2 years later, further worsening of biapical infiltrates with images of honeycombing, appearance of new subpleural parenchymal consolidations, and greater lung volume loss with more architectural distortion of the parenchyma were seen (Figure 7). We performed a thoracic ultrasound (TUS) in the left hemithorax wall (lateral region from the anterior axillary line to the posterior axillary line) that showed visceral pleural thickening (Figure 8).


Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias.

Portillo K, Guasch I, Becker C, Andreo F, Fernández-Figueras MT, Ramirez Ruz J, Martinez-Barenys C, García-Reina S, Lopez de Castro P, Sansano I, Villar A, Ruiz-Manzano J - Case Rep Pulmonol (2015)

Case  2. (a) Axial HRCT scan shows very discrete lineal opacities in both lung apices. (b) Axial HRCT scan at the same level compared to (a) two years and two months later shows a loss of volume and prominent parenchymal fibrotic infiltrates. (c) Axial HRCT image at the level of carina reveals lung architectural distortion, as well as the presence of pleural thickening and subpleural parenchymal opacities.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4563085&req=5

fig7: Case  2. (a) Axial HRCT scan shows very discrete lineal opacities in both lung apices. (b) Axial HRCT scan at the same level compared to (a) two years and two months later shows a loss of volume and prominent parenchymal fibrotic infiltrates. (c) Axial HRCT image at the level of carina reveals lung architectural distortion, as well as the presence of pleural thickening and subpleural parenchymal opacities.
Mentions: However, the second HRCT, performed during the period in which the patient experienced the spontaneous pneumothorax, revealed that the upper linear opacities had progressed to pulmonary infiltrates of fibrotic appearance. Also evident was a thickening of the visceral pleura, clearly visible due to the adjacent pneumothorax (Figure 6). Finally, in a HRCT made 2 years later, further worsening of biapical infiltrates with images of honeycombing, appearance of new subpleural parenchymal consolidations, and greater lung volume loss with more architectural distortion of the parenchyma were seen (Figure 7). We performed a thoracic ultrasound (TUS) in the left hemithorax wall (lateral region from the anterior axillary line to the posterior axillary line) that showed visceral pleural thickening (Figure 8).

Bottom Line: The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections.That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team.We present the first reported cases in Spain.

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.

ABSTRACT
Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.

No MeSH data available.


Related in: MedlinePlus