• Users Online: 63
  • Print this page
  • Email this page


 
 
Table of Contents
CASE REPORT
Year : 2018  |  Volume : 20  |  Issue : 2  |  Page : 104-106

The case of idiopathic pleuroparenchymal fibroelastosis diagnosed by transbronchial biopsy


1 Department of Chest Diseases, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey
2 Department of Thoracic Surgery, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey
3 Department of Pathology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey

Date of Web Publication31-Aug-2018

Correspondence Address:
Prof. Muhammet Sayan
Department of Thoracic Surgery, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Avsar Campus, Onikisubat 46100 Kahramanmaras
Turkey
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejop.ejop_12_18

Rights and Permissions
  Abstract 


Pleuroparenchymal fibroelastosis (PPFE) is claasified in idiopathic interstitial pneumonitis group is rarely seen. Etiology and pathophysiology of this entity are not completely understood. The prognosis is poor, and there is no effective treatment except for lung transplantation. Here, we presented a case of PPFE diagnosed by transbronchial biopsy through fiberoptic bronchoscopy and its histopathological and clinicoradiological features.

Keywords: Idiopathic, pleuroparenchymal fibroelastosis, transbronchial biopsy


How to cite this article:
Arpag H, Sayan M, Atilla N, Bozkus F, Kahraman H, Bahar AY, Tokur M. The case of idiopathic pleuroparenchymal fibroelastosis diagnosed by transbronchial biopsy. Eurasian J Pulmonol 2018;20:104-6

How to cite this URL:
Arpag H, Sayan M, Atilla N, Bozkus F, Kahraman H, Bahar AY, Tokur M. The case of idiopathic pleuroparenchymal fibroelastosis diagnosed by transbronchial biopsy. Eurasian J Pulmonol [serial online] 2018 [cited 2018 Dec 17];20:104-6. Available from: http://www.eurasianjpulmonol.com/text.asp?2018/20/2/104/240172




  Introduction Top


Pleuroparenchymal fibroelastosis (PPFE) is an idiopathic interstitial pneumonitis. This rare entity characterized by fibroelastic the pleural and subpleural lung parenchymal thickening particularly located in the upper lobes of lungs. The prognosis is poor and there is no effective treatment except for lung transplantation. There is a very few information about etiology and pathophysiology of PPFE.[1],[2],[3] Here, we presents a case of PPFE.


  Case Report Top


A 54-year-old, nonsmoker farmer male admitted to clinic with cough and for dyspnea complaints. He had no known respiratory, rheumatologic disease, or tuberculosis. There was general decreased respiratory sound on thorax auscultation. Clubbing and pretibial edema were not detected. The oxygen saturation was measured as 98% with finger probe in the room air. His body mass index was 18.1 kg/m 2. Right tracheal deviation, bilateral apical fibrotic areas, increment in bilateral reticular trails, and blunting in bilateral costophrenic angle were seen on chest X-ray and thorax computerized tomography [Figure 1]. Mix pattern was detected on pulmonary function test (forced vital capacity; 2.25 L [60%], forced expiratory volume1; 1.23 L [41%], and Tiffeneau index; 55%). There was no hypercapnia in the arterial blood gas and the oxygen pressure was 82 mmHg. He had no known occupational-environmental exposure and familial lung diseases and his rhematological markers were negative. Informed consent form included intervention and publishability of his medical records was obtained from the patient then fiberoptic bronchoscopy and transbronchial biopsy was performed. The results of acid-fast bacilli tests and tuberculosis culture (Löwenstein–Jensen medium) from sputum samples were negative. Final pathology report revealed that alveolar collapse, thickening of alveolar septum, and peribronchial hyalinization were observed. Elastic fiber accumulation was detected in the alveolar septum and air spaces with the histochemically applied orcein stain [Figure 2] and [Figure 3]. The patient was diagnosed as idiopathic PPFE according the clinicoradiological and histopathological findings.
Figure 1: Chest X-ray and computed tomography revealed; right tracheal deviation, bilateral apical fibrotic areas, increment in bilateral reticular trails, and blunting in bilateral costophrenic angles

Click here to view
Figure 2: Markedly fibrosis and increased chronic inflammatory cells in the interstitial area (H and E, ×200)

Click here to view
Figure 3: Positive reaction with orcein stain in elastic fibers (dark red)

Click here to view



  Discussion Top


PPFE is a rare condition and characterized by fibroelastic thickening in the pleura and subpleural lung parenchyma especially in the upper lobes.[1] It was first reported in 2004 by Frankel et al.[2] In recent years, PPFE was included into idiopathic interstitial pneumonias (IIP) group by the international multidisciplinary classification.[1],[2],[3] There is a very few information about etiology and pathophysiology of PPFE. Although PPFE is been usually idiopathic, it may be associated connective tissue diseases, lung transplantation, bone marrow transplantation, or genetic condition in some cases. Our case was considered as idiopathic. Clinical manifestations are vary but the most common complaints of patients with PPFE are dypsnea and nonproductive cough.[4] Our patient had exertional dyspnea and dry cough symptoms. Thorax computerized tomography findings of PPFE are usually characterized by bilateral fibrosis, bronchiectasis, and subpleural reticular pattern located especially upper zones.[1],[2] Similar radiological findings were detected in our case. Usually, restrictive pattern is observed in pulmonary functions tests.[5] However, the combination of restriction and obstruction (mix pattern) was seen in ours. Histopathologically, it is characterized by collagen fibrosis, septal elastosis, and elastin accumulation, especially in subpleural space and intraalveolar space.[3],[6],[7],[8] Elastin accumulation was shown through the orcein stain in tissue sample taken through transbronchial biopsy in our case. Although the definite diagnosis of PPFE is surgical lung biopsy, it is not preferred by physicians due to the bad general condition of these patients and complications of operation, especially pneumothorax and prolonged air leak.[6] Therefore, less invasive diagnostic procedures such as transthoracic biopsy, bronchoscopic transbronchial lung biopsy, or transbronchial crio biopsy which is used in increasingly day-by-day are recommended for diagnosis of diseases.[1],[2],[3],[6] Despite immunosuppressive therapy is used in some patients, definitive treatment currently known is lung transplantation.[7] Bronchodilator therapy is started for airway obstruction to our case and he referred to lung transplantation center.

The less invasive procedures should be preferred for diagnosis of PPFE which is classified in IIP group so possible complication can be prevented.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Piciucchi S, Tomassetti S, Casoni G, Sverzellati N, Carloni A, Dubini A, et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis. Respir Res 2011;12:111.  Back to cited text no. 1
    
2.
Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: Description of a novel clinicopathologic entity. Chest 2004;126:2007-13.  Back to cited text no. 2
    
3.
Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis: A spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377-85.  Back to cited text no. 3
    
4.
Travis WD, Costabel U, Hansell DM, King TE Jr., Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society Statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:733-48.  Back to cited text no. 4
    
5.
Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S, Enomoto N, et al. Idiopathic pleuroparenchymal fibroelastosis: Consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med 2012;12:72.  Back to cited text no. 5
    
6.
Kushima H, Hidaka K, Ishii H, Nakao A, On R, Kinoshita Y, et al. Two cases of pleuroparenchymal fibroelastosis diagnosed with transbronchial lung biopsy. Respir Med Case Rep 2016;19:71-3.  Back to cited text no. 6
    
7.
Redondo MT, Melo N, Mota PC, Jesus JM, Moura CS, Guimarães S, et al. Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity. Rev Port Pneumol (2006) 2015;21:41-4.  Back to cited text no. 7
    
8.
Watanabe K, Nagata N, Kitasato Y, Wakamatsu K, Nabeshima K, Harada T, et al. Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Investig 2012;50:88-97.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed237    
    Printed11    
    Emailed0    
    PDF Downloaded54    
    Comments [Add]    

Recommend this journal