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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) Initial chest radiograph after the diagnosis of breast cancer and before chemotherapy and radiotherapy treatment was normal. (b), (c), and (d) Chest radiographs evolution along two-year and two-month interval reveal progressive worsening. Notice progressive gradual loss of lung volume at the expense of the upper lobes, which is manifested by superior shrinkage and distortion of the pulmonary hila, as well as biapical pleural thickening. (e) Lateral chest radiograph shows flattening of the chest, observed by the decreased anteroposterior diameter of the rib cage (arrow).
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fig5: Case  2. (a) Initial chest radiograph after the diagnosis of breast cancer and before chemotherapy and radiotherapy treatment was normal. (b), (c), and (d) Chest radiographs evolution along two-year and two-month interval reveal progressive worsening. Notice progressive gradual loss of lung volume at the expense of the upper lobes, which is manifested by superior shrinkage and distortion of the pulmonary hila, as well as biapical pleural thickening. (e) Lateral chest radiograph shows flattening of the chest, observed by the decreased anteroposterior diameter of the rib cage (arrow).

Mentions: Case  2. The patient was a 40-year-old female without history of smoking and with a history of exposure to birds. She had a history of childhood asthma and a family history of breast cancer. She was diagnosed at 37 years with bilateral invasive ductal carcinoma with axillary lymph node metastasis during pregnancy. She received neoadjuvant chemotherapy before surgery with Adriamycin and cyclophosphamide followed by docetaxel. She underwent bilateral mastectomy with dissection of axillary lymph nodes and received adjuvant chemotherapy with 4 cycles of cyclophosphamide, methotrexate, and fluorouracil with concomitant radiotherapy afterwards. The initial chest radiography after the diagnosis of breast cancer was normal. However, in the follow-up chest radiographs, a progressive loss of lung volume at the expense of the upper lobes was noticed, which manifested by superior shrinkage and distortion of the pulmonary hila, and the appearance of coarse irregular linear opacities at higher fields and biapical pleural thickening with linear opacities of scar appearance in the neighboring lung parenchyma (see Figure 5). 15 months after radiotherapy, the patient presented with acute dyspnea and chest pain. She was diagnosed with right spontaneous pneumothorax that required thorax drainage.


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) Initial chest radiograph after the diagnosis of breast cancer and before chemotherapy and radiotherapy treatment was normal. (b), (c), and (d) Chest radiographs evolution along two-year and two-month interval reveal progressive worsening. Notice progressive gradual loss of lung volume at the expense of the upper lobes, which is manifested by superior shrinkage and distortion of the pulmonary hila, as well as biapical pleural thickening. (e) Lateral chest radiograph shows flattening of the chest, observed by the decreased anteroposterior diameter of the rib cage (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig5: Case  2. (a) Initial chest radiograph after the diagnosis of breast cancer and before chemotherapy and radiotherapy treatment was normal. (b), (c), and (d) Chest radiographs evolution along two-year and two-month interval reveal progressive worsening. Notice progressive gradual loss of lung volume at the expense of the upper lobes, which is manifested by superior shrinkage and distortion of the pulmonary hila, as well as biapical pleural thickening. (e) Lateral chest radiograph shows flattening of the chest, observed by the decreased anteroposterior diameter of the rib cage (arrow).
Mentions: Case  2. The patient was a 40-year-old female without history of smoking and with a history of exposure to birds. She had a history of childhood asthma and a family history of breast cancer. She was diagnosed at 37 years with bilateral invasive ductal carcinoma with axillary lymph node metastasis during pregnancy. She received neoadjuvant chemotherapy before surgery with Adriamycin and cyclophosphamide followed by docetaxel. She underwent bilateral mastectomy with dissection of axillary lymph nodes and received adjuvant chemotherapy with 4 cycles of cyclophosphamide, methotrexate, and fluorouracil with concomitant radiotherapy afterwards. The initial chest radiography after the diagnosis of breast cancer was normal. However, in the follow-up chest radiographs, a progressive loss of lung volume at the expense of the upper lobes was noticed, which manifested by superior shrinkage and distortion of the pulmonary hila, and the appearance of coarse irregular linear opacities at higher fields and biapical pleural thickening with linear opacities of scar appearance in the neighboring lung parenchyma (see Figure 5). 15 months after radiotherapy, the patient presented with acute dyspnea and chest pain. She was diagnosed with right spontaneous pneumothorax that required thorax drainage.

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