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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

Histological features of Case  1 (surgical lung biopsy specimen). (a) Microscopic image showing sparsely cellular dense connective tissue with small vessels. Hematoxylin-eosin (HE) 100x. (b) At high power inset in area of dense fibrosis with multiple capillaries, without inflammatory infiltrate appearance. HE 250x. (c) Microscopic image shows the presence of abundant elastic fibers irregularly grouped. Orcein stain 100x. (d) Microscopic appearance of the image (b), visualized by fluorescence. Elastic fibers, which shine for eosin autofluorescence, can be observed. Fluorescence microscopy 250x.
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fig2: Histological features of Case  1 (surgical lung biopsy specimen). (a) Microscopic image showing sparsely cellular dense connective tissue with small vessels. Hematoxylin-eosin (HE) 100x. (b) At high power inset in area of dense fibrosis with multiple capillaries, without inflammatory infiltrate appearance. HE 250x. (c) Microscopic image shows the presence of abundant elastic fibers irregularly grouped. Orcein stain 100x. (d) Microscopic appearance of the image (b), visualized by fluorescence. Elastic fibers, which shine for eosin autofluorescence, can be observed. Fluorescence microscopy 250x.

Mentions: After 3 months, the patient developed progressive clinical deterioration with hypoxemia and oxygen desaturation during a 6-minute walk test (6 MWT) with minimal saturation of 81%. Therefore, he was treated with supplemental oxygen and oral corticosteroids (1 mg/kg/day with progressive dose reduction). SLB was performed a month after initiating the treatment with right apical pneumothorax as a complication. The biopsy revealed marked subpleural thickening and homogenous and extensive areas of fibrosis. Dense elastic fibers were seen in the subpleural area and adjacent parenchyma on elastic stain (Figure 2). After having made the diagnosis by SLB, it was decided to reexamine the histological sample from the cryobiopsy and make the Weigert Van Gieson stain, observing the elastic fibers herein (Figure 3).


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)

Histological features of Case  1 (surgical lung biopsy specimen). (a) Microscopic image showing sparsely cellular dense connective tissue with small vessels. Hematoxylin-eosin (HE) 100x. (b) At high power inset in area of dense fibrosis with multiple capillaries, without inflammatory infiltrate appearance. HE 250x. (c) Microscopic image shows the presence of abundant elastic fibers irregularly grouped. Orcein stain 100x. (d) Microscopic appearance of the image (b), visualized by fluorescence. Elastic fibers, which shine for eosin autofluorescence, can be observed. Fluorescence microscopy 250x.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig2: Histological features of Case  1 (surgical lung biopsy specimen). (a) Microscopic image showing sparsely cellular dense connective tissue with small vessels. Hematoxylin-eosin (HE) 100x. (b) At high power inset in area of dense fibrosis with multiple capillaries, without inflammatory infiltrate appearance. HE 250x. (c) Microscopic image shows the presence of abundant elastic fibers irregularly grouped. Orcein stain 100x. (d) Microscopic appearance of the image (b), visualized by fluorescence. Elastic fibers, which shine for eosin autofluorescence, can be observed. Fluorescence microscopy 250x.
Mentions: After 3 months, the patient developed progressive clinical deterioration with hypoxemia and oxygen desaturation during a 6-minute walk test (6 MWT) with minimal saturation of 81%. Therefore, he was treated with supplemental oxygen and oral corticosteroids (1 mg/kg/day with progressive dose reduction). SLB was performed a month after initiating the treatment with right apical pneumothorax as a complication. The biopsy revealed marked subpleural thickening and homogenous and extensive areas of fibrosis. Dense elastic fibers were seen in the subpleural area and adjacent parenchyma on elastic stain (Figure 2). After having made the diagnosis by SLB, it was decided to reexamine the histological sample from the cryobiopsy and make the Weigert Van Gieson stain, observing the elastic fibers herein (Figure 3).

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