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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

Surgical lung biopsy of the right upper lobe of Case  2. (a) The alveolar structures are completely obliterated due to accumulation of elastotic material in the wall. HE 100x. (b) At higher magnification, the presence of a very small lymphocytic infiltration is showed. (c) Using the Van Gieson stain, the elastotic content of the wall is observed and the alveolar lumen is replaced by connective tissue. (d) Using autofluorescence, the presence of numerous elastic fibbers is observed in the same field of Figure 9(b).
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fig9: Surgical lung biopsy of the right upper lobe of Case  2. (a) The alveolar structures are completely obliterated due to accumulation of elastotic material in the wall. HE 100x. (b) At higher magnification, the presence of a very small lymphocytic infiltration is showed. (c) Using the Van Gieson stain, the elastotic content of the wall is observed and the alveolar lumen is replaced by connective tissue. (d) Using autofluorescence, the presence of numerous elastic fibbers is observed in the same field of Figure 9(b).

Mentions: In the blood analysis, the autoimmunity was negative and serological exams showed positive test results for Aspergillus and avian precipitins. PFT showed severe restrictive pattern and an increase of the relationship between residual volume (RV) and TLC (Table 1). The patient underwent breast reconstruction; therefore, SLB was delayed for seven months. Postoperatively, she presented another right loculated pneumothorax that resolved spontaneously. The pathology revealed subpleural parenchymal fibroelastosis with extension into adjacent alveolar walls predominantly in the upper lobes (Figures 9 and 10). From an oncological point of view, by not showing recurrence at 5 years after diagnosis of breast cancer, the patient was derived to the LT list and pulmonary rehabilitation with improvement of dyspnea score (NYHA class I).


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)

Surgical lung biopsy of the right upper lobe of Case  2. (a) The alveolar structures are completely obliterated due to accumulation of elastotic material in the wall. HE 100x. (b) At higher magnification, the presence of a very small lymphocytic infiltration is showed. (c) Using the Van Gieson stain, the elastotic content of the wall is observed and the alveolar lumen is replaced by connective tissue. (d) Using autofluorescence, the presence of numerous elastic fibbers is observed in the same field of Figure 9(b).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig9: Surgical lung biopsy of the right upper lobe of Case  2. (a) The alveolar structures are completely obliterated due to accumulation of elastotic material in the wall. HE 100x. (b) At higher magnification, the presence of a very small lymphocytic infiltration is showed. (c) Using the Van Gieson stain, the elastotic content of the wall is observed and the alveolar lumen is replaced by connective tissue. (d) Using autofluorescence, the presence of numerous elastic fibbers is observed in the same field of Figure 9(b).
Mentions: In the blood analysis, the autoimmunity was negative and serological exams showed positive test results for Aspergillus and avian precipitins. PFT showed severe restrictive pattern and an increase of the relationship between residual volume (RV) and TLC (Table 1). The patient underwent breast reconstruction; therefore, SLB was delayed for seven months. Postoperatively, she presented another right loculated pneumothorax that resolved spontaneously. The pathology revealed subpleural parenchymal fibroelastosis with extension into adjacent alveolar walls predominantly in the upper lobes (Figures 9 and 10). From an oncological point of view, by not showing recurrence at 5 years after diagnosis of breast cancer, the patient was derived to the LT list and pulmonary rehabilitation with improvement of dyspnea score (NYHA class I).

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