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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  1. (a) Microscopic examination of explanted lung of Case  1 (right upper lobe). The increase of elastic fibers and abrupt transition to normal parenchyma is evident. HE 200x. (b) The involved areas show collapse of alveolar spaces by interstitial material that corresponds to elastic fibers. Scant lymphocyte infiltrate. HE 400x. (c) Van Gieson stain demonstrates abnormal elastic fibers in the thickened interstitium.
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fig4: Case  1. (a) Microscopic examination of explanted lung of Case  1 (right upper lobe). The increase of elastic fibers and abrupt transition to normal parenchyma is evident. HE 200x. (b) The involved areas show collapse of alveolar spaces by interstitial material that corresponds to elastic fibers. Scant lymphocyte infiltrate. HE 400x. (c) Van Gieson stain demonstrates abnormal elastic fibers in the thickened interstitium.

Mentions: The patient underwent bilateral lung transplantation (LT) at 14 months after diagnosis. Besides pleural fibroelastosis in the upper lobes (Figure 4), the pathological examination of the explant describes a pattern of nonspecific interstitial pneumonia (NSIP) in the lower lobes. Currently, the patient has 24-month survival after LT.


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  1. (a) Microscopic examination of explanted lung of Case  1 (right upper lobe). The increase of elastic fibers and abrupt transition to normal parenchyma is evident. HE 200x. (b) The involved areas show collapse of alveolar spaces by interstitial material that corresponds to elastic fibers. Scant lymphocyte infiltrate. HE 400x. (c) Van Gieson stain demonstrates abnormal elastic fibers in the thickened interstitium.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig4: Case  1. (a) Microscopic examination of explanted lung of Case  1 (right upper lobe). The increase of elastic fibers and abrupt transition to normal parenchyma is evident. HE 200x. (b) The involved areas show collapse of alveolar spaces by interstitial material that corresponds to elastic fibers. Scant lymphocyte infiltrate. HE 400x. (c) Van Gieson stain demonstrates abnormal elastic fibers in the thickened interstitium.
Mentions: The patient underwent bilateral lung transplantation (LT) at 14 months after diagnosis. Besides pleural fibroelastosis in the upper lobes (Figure 4), the pathological examination of the explant describes a pattern of nonspecific interstitial pneumonia (NSIP) in the lower lobes. Currently, the patient has 24-month survival after LT.

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