0
ONLINE EXCLUSIVES
Chest Imaging and Pathology for Clinicians |

Chronic Cough and Bilateral Pneumothoraces in a Nonsmoker FREE TO VIEW

Sarah L. O’Beirne, MD, PhD; Joanna G. Escalon, MD; Jordan E. Arkin, MD; Brendon M. Stiles, MD; Robert J. Kaner, MD; Alan C. Legasto, MD; Navneet Narula, MD; Thomas C. King, MD
Author and Funding Information

CORRESPONDENCE TO: Sarah L. O’Beirne, MD, PhD, Division of Pulmonary and Critical Care Medicine, 525 East 68th St, New York, NY 10065


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(2):e49-e55. doi:10.1016/j.chest.2015.10.070
Text Size: A A A
Published online

An 82-year-old Japanese nonsmoking man presented with persistent dry cough and small left apical pneumothorax. High resolution CT scan of the chest demonstrated bilateral upper lobe pleuroparenchymal thickening and architectural distortion. Serial imaging revealed mild progression and development of small bilateral pneumothoraces, and pneumomediastinum. A surgical lung biopsy was required to confirm the diagnosis.

Figures in this Article

An 82-year-old Japanese man was assessed in pulmonary clinic following the discovery of a small left apical pneumothorax on imaging (Fig 1A) performed because of persistent dry cough and 13.6-kg weight loss during the preceding year. His exercise tolerance was normal, and he was not experiencing chest pain. Past medical, family, and medication histories were noncontributory. He was a lifelong never smoker working in consultancy without known occupational or environmental exposures. On clinical examination vital signs and oxygen saturation were normal. He was thin with a body mass index of 15.7 kg/m2, without clubbing or lymphadenopathy. Respiratory, cardiovascular, abdominal, and rheumatologic examinations were unremarkable.

Figure 1
Figure Jump LinkFigure 1 Initial chest radiograph (A) demonstrates a small left apical pneumothorax and bilateral subpleural reticulation, most pronounced in the upper lobes. Initial noncontrast high-resolution computed tomography of the chest in the coronal plane (B), axial supine inspiratory view of the apices (C), and axial prone inspiratory view of the bases (D) demonstrates a small left hydropneumothorax and bilateral upper zone predominant irregular pleuroparenchymal thickening, architectural distortion, traction bronchiectasis, and tracheomegaly. There is relative sparing of the lower lobes, which demonstrate nonspecific subpleural reticulations, which can be seen in the setting of usual interstitial pneumonia or idiopathic pulmonary fibrosis. No evidence of honeycombing, bullous disease, or mosaic attenuation is identified.Grahic Jump Location

Laboratory testing including autoantibodies, hypersensitivity pneumonitis, and mold allergen panels were negative. Pulmonary function tests revealed restrictive spirometry, reduced lung volumes, and normal diffusion capacity of the lung for carbon monoxide. CT scan of the chest demonstrated a small left-sided pneumothorax with bilateral upper lobe predominant irregular pleuroparenchymal thickening, architectural distortion, and traction bronchiectasis (Fig 1B-D).

Bronchoscopy revealed dilated airways with thick secretions. The differential cell count of the bronchoalveolar lavage was 98% macrophages and 2% lymphocytes. Microbiologic analysis of bronchoalveolar lavage fluid including bacterial and viral culture was negative. Mycobacterial culture grew Mycobacterium avium-intracellulare complex. Sparse Candida albicans, Aspergillus species, and Penicillium species were isolated on fungal culture. Right lower lobe transbronchial biopsies demonstrated alveolar parenchyma and bronchial mucosa with focal nonspecific chronic inflammation and rare nonnecrotizing granulomata. Based on these findings, itraconazole and prednisone were commenced with improvement in cough although pulmonary function tests continued to decline.

During the following 3 months he reported stable symptoms, although imaging demonstrated mild progression of the upper lobe predominant subpleural reticulation, interlobular septal thickening, and variable, small bilateral pneumothoraces, ultimately with increase in size of the right pneumothorax, persistence of the left pneumothorax, and development of pneumomediastinum (Fig 2), perhaps related to the pneumothorax. Given the bilateral nature of the pneumothoraces, he was considered at high risk of decompensation, so proceeded to a video-assisted thoracoscopy with right upper and middle lobe wedge biopsies and talc pleurodesis.

Figure 2
Figure Jump LinkFigure 2 Noncontrast high-resolution computed tomography of the chest in the coronal plane (A), axial supine inspiratory view of the apices (B), inferior upper lobes (C), and bases (D) at 5-month follow-up demonstrates new pneumomediastinum extending into the soft tissues of the neck and inferiorly to the level of the diaphragm as well as a new right apical pneumothorax and persistent left apical pneumothorax. There has been interval mild progression of the upper lobe predominant subpleural reticulation. Interlobular septal thickening is present, and the lung bases are unchanged from prior examination. E, Sagittal reformatted views demonstrate anteroposterior flattening of the chest wall.Grahic Jump Location

Surgical lung biopsies (SLB) of the right upper and middle lobes revealed pleural fibrosis and prominent subpleural alveolar fibrosis and elastosis obliterating the lung architecture with partial occlusion of the pulmonary arteries and veins. The fibroelastosis extended along the interlobular septa and involved the bronchovascular bundles. Elastic staining highlighted the elastosis (Fig 3).

Figure 3
Figure Jump LinkFigure 3 Surgical lung biopsy with low-power (×2) magnification with hematoxylin and eosin stain depicting pleural (large arrows pointing to pleura) and subpleural fibrosis and elastosis (A). There is subpleural accentuation of fibroelastosis with an abrupt transition to normal lung tissue (small arrows), and higher-power (×10) magnification with elastic staining demonstrating fragmented elastic fibers and prominent subpleural alveolar septal elastosis obliterating the lung architecture (B). Vascular involvement was also present, with a small muscular artery with recanalized thrombus (short arrow) and medial hyperplasia (long arrow) seen (×10) (C), and another vessel with luminal obliteration and fragmented elastic fibers (×10) (D).Grahic Jump Location

What is the diagnosis?

Diagnosis: Pleuroparenchymal fibroelastosis (PPFE)

Clinical Discussion

PPFE is an upper lobe predominant progressive fibrosis with specific pathologic findings including visceral pleural thickening, subpleural elastosis, and intraalveolar collagenous fibrosis. It is an uncommon condition first described in the Japanese literature in 1992 and subsequently reported in English-language literature in 2004. PPFE is now recognized by the American Thoracic Society (ATS)/European Respiratory Society (ERS) in their most recent statement on idiopathic interstitial pneumonia in the category of rare idiopathic interstitial pneumonia.

The etiology of PPFE is unknown, with most cases considered idiopathic. Familial cases of PPFE and other interstitial lung disease have been reported, and the condition may be more common in the Japanese, suggesting genetic predisposition plays a role in pathogenesis. PPFE has been associated with bone marrow transplantation and lung transplantation,, together accounting for more than 50% of cases. Autoantibodies are present in 8%, and recurrent chest infections have been reported in 14% to 50% of all cases. Various organisms have been isolated including Aspergillus specis and Mycobacterium avium-intracellulare complex. Coexistent allergic bronchopulmonary aspergillosis and positive Aspergillus species, mold, and avian precipitins have been described, leading authors to postulate that repeated inflammatory damage leads to intraalveolar fibroelastosis and eventually to PPFE.

Most patients are between 49 and 57 years old at diagnosis, with both sexes affected equally. No active smokers have been reported, and the majority of cases occur in never smokers (62%–75%). Dyspnea (79%) and dry cough (52%) are the most common presenting symptoms. Twenty percent experience weight loss, and recurrent chest infections are frequent. Spontaneous pneumothorax occurs in 30% of cases; these are generally small and often recurrent or bilateral. Pneumomediastinum is also common. Clubbing is unusual, most patients are thin, and 50% have crackles. Anteroposterior flattening of the chest wall may occur. Pulmonary function test abnormalities are most commonly consistent with restrictive lung disease with reduced lung volumes and diffusion capacity of the lung for carbon monoxide. As the disease progresses, the forced vital capacity may drop by up to 20% a year.

There are no specific agreed diagnostic criteria, with diagnosis made based on a distinct array of clinical, radiologic, and pathologic findings discussed in detail subsequently. Diagnosis has traditionally required a lung biopsy with pathologic confirmation of PPFE, but as disease definition improves, more cases are being diagnosed noninvasively. It is proposed that in this circumstance the term consistent with PPFE be used.,, PPFE is generally slowly progressive and unresponsive to steroids and immunosuppressive medications., Management should focus on best supportive care, including oxygen therapy, pulmonary rehabilitation, and symptom palliation. There have been reports of successful lung transplantation. The median overall survival is 11 years.

Radiologic Discussion

High-resolution computed tomography scanning is the preferred method of imaging in interstitial lung disease, and finding classic radiologic features of a disease can obviate the need for SLB. Classic high-resolution computed tomography findings in PPFE include bilateral upper and midzone predominant irregular pleuroparenchymal thickening and architectural distortion, with most cases demonstrating subpleural fibrosis of varying severity. Small upper zone predominant foci of consolidation and areas of traction bronchiectasis may be present. When these features are present, a radiologic diagnosis of PPFE should be heavily favored. The presence of lower lobe changes does not exclude the diagnosis of PPFE, and interstitial fibrosis may extend to the lower lobes, particularly as the condition progresses. Proposed radiologic criteria for definite PPFE include upper lobe pleural thickening and subpleural fibrosis and minimal or absent lower lobe involvement,, whereas imaging features consistent with PPFE are the presence of upper lobe pleural thickening and subpleural fibrosis with changes not localized to the upper lobes or additional features of coexistent disease.,

A less specific feature of PPFE is platythorax, defined as a significant reduction of the ratio of anteroposterior diameter of the thorax to the transverse diameter of the thorax, with deformity increasing as disease progresses. Platythorax was noted in this case, as was tracheomegaly. Anteroposterior flattening of the chest wall was noted in this case, as was tracheomegaly. Although there is no recognized association between PPFE and tracheobronchomegaly, it has been described in association with bronchiectasis.

When upper lobe pleural thickening and subpleural fibrosis are present, but not the predominant distribution and/or there are features of coexistent disease, the radiologic findings are suggestive of PPFE, with a possible coexistent additional interstitial lung disease. The most common coexistent radiologic or pathologic patterns are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia. For example, Oda et al described a series of cases meeting radiologic criteria for PPFE in addition to having a UIP pattern on SLB. By current guidelines, these cases are considered possible idiopathic pulmonary fibrosis (IPF). However, they appear to represent a distinct disease entity with increased incidence of pneumothorax and pneumomediastinum, more in keeping with PPFE rather than IPF or UIP, with the authors suggesting that they should be classified as PPFE. Interestingly, two similar cases in a series of 12 reported by Reddy et al had progression of lower lobe UIP pattern fibrosis with stability of the upper PPFE change on serial follow-up.

Differential diagnosis of the imaging features includes late stage hypersensitivity pneumonitis, apical cap, connective tissue disease, advanced fibrosing sarcoidosis, drug-induced lung disease, radiation, and prior TB. Although hypersensitivity pneumonitis also affects the upper lobes, it is bronchiolocentric, unlike PPFE. Apical cap is a localized lesion of subpleural fibrosis limited to the apices and lacks the more diffuse parenchymal component seen in PPFE. Additionally it tends to be asymptomatic and nonprogressive, and is found in older cigarette smokers. Although healed TB infection can cause apical scarring with superior hilar retraction, this is nonprogressive and there are often other indications of prior granulomatous exposure such as calcified granulomas. These additional differential considerations are usually distinguishable by patient history (eg, radiation and drug-induced lung disease) or associated clinical and imaging findings.

Pathologic Discussion

PPFE has unique pathologic features including upper lobe predominant pleural and subpleural fibrosis and prominent elastosis with short and curled, randomly oriented elastic fibers. In contrast to most other pulmonary fibrotic conditions in which collagen fibers predominate, twice as much elastin is seen in PPFE compared with IPF. A sharp cutoff between the thickened abnormal tissue and normal lung parenchyma is seen with sparing of parenchyma distant from the pleura. Mild patchy lymphocytic infiltrates and few fibroblastic foci are present. Cysts and honeycomb change are not seen in PPFE, and unlike IPF, the condition is temporally homogeneous. Additionally, partial occlusion of pulmonary arteries and veins, acute or organizing thromboemboli, and occasionally granulomatous inflammation have been observed. In cases in which intraalveolar fibrosis as described previously is present, but is not associated with pleural fibrosis or the pathologic abnormalities are not subpleural or upper lobe predominant, the findings are considered consistent with PPFE. Some cases of PPFE, particularly those associated with bone marrow transplantation and lung transplantation show foci with histologic features of acute lung injury including diffuse alveolar damage becoming confluent with PPFE, suggesting that acute lung injury may precede PPFE.,,

Proposed histologic criteria for definite PPFE include fibrosis of upper lobe visceral pleura, prominent, homogeneous subpleural intraalveolar fibrosis and septal elastosis with sparing of the lung parenchyma remote from the pleura, few fibroblastic foci, and minimal lymphocytic infiltrates., The term consistent with PPFE is preferred when intraalveolar fibrosis is present but significant pleural disease is absent, fibrosis occurs distant from the pleura, or fibrosis is not present in the upper lobes.,

As previously discussed, PPFE may be seen in association with other radiologic and pathologic patterns including UIP and fibrotic nonspecific interstitial pneumonia. The coexistence of these conditions stresses the importance of adequate sampling at the time of SLB.

The differential diagnosis of coexistent pleural and parenchymal fibrosis includes asbestosis, connective tissue disease associated lung disease, advanced fibrotic sarcoidosis, or drug-induced lung disease. Also, intraalveolar fibroelastosis is not unique to PPFE, and differential diagnosis includes radiotherapy-related changes, drug-induced lung disease, connective tissue disease, certain inhalational injuries, and apical cap. The predominance of elastic fibers in PPFE aids in distinguishing it from other conditions. Given its subpleural distribution, PPFE may be confused with UIP (either IPF or secondary to known causes); however, PPFE is temporally homogeneous, has few fibroblastic foci, and honeycomb change is absent. Also UIP tends to be lower lobe predominant at least initially. Correlation with clinical, radiologic, and serologic parameters is needed for differentiating PPFE from all the other conditions.

PPFE is a rare and probably underrecognized condition, which is now classified by ATS/ERS as a rare idiopathic interstitial pneumonia. This case illustrates a classic presentation of the disease in a Japanese patient with bilateral pneumothoraces, confirmed by SLB at the time of pleurodesis. Sadly, his disease progressed and he succumbed to it within 3 years of presentation. As PPFE is better characterized, more patients are being diagnosed noninvasively without the necessity for pathologic confirmation by SLB. Currently, there is no specific therapy for PPFE, and management should focus on best supportive care, although successful lung transplantation has been described in select cases.

Author contributions: S. L. O’B. cared for patient, and prepared, reviewed, and submitted manuscript; J. G. E. and A. C. L. prepared radiology images and prepared and reviewed manuscript; J. E. A. and N. N. prepared pathology images and prepared and reviewed manuscript; B. M. S. and T. C. K. cared for patient, and prepared and reviewed manuscript; and R. J. K. prepared and reviewed manuscript.

Financial/nonfinancial disclosures: The authors have reported to CHEST the following: R. C. K. serves on adjudication committees and provides nonpromotional speaking or consulting for the following companies on pulmonary fibrosis (all Boehringer Ingelheim, Celgene, Genentech/Roche, Gilead, Medimmune/Astra Zeneca). None declared (S. L. O’B., J. G. E., J. E. A., B. M. S., A. C. L., N. N., T. C. K.).

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Amitani R. .Miimi A. .Kuse F. . Idiopathic pulmonary upper lobe fibrosis (IPUF). Kokyu. 1992;11:693-699 [PubMed]journal
 
Frankel S.K. .Cool C.D. .Lynch D.A. .Brown K.K. . Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126:2007-2013 [PubMed]journal. [CrossRef] [PubMed]
 
Travis W.D. .Costabel U. .Hansell D.M. . ATS/ERS Committee on Idiopathic Interstitial Pneumoniaset 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;188:733-748 [PubMed]journal. [CrossRef] [PubMed]
 
Kusagaya H. .Nakamura Y. .Kono M. .et al Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 2012;12:72- [PubMed]journal. [CrossRef] [PubMed]
 
von der Thusen J.H. .Hansell D.M. .Tominaga M. .et al Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011;24:1633-1639 [PubMed]journal. [CrossRef] [PubMed]
 
Hirota T. .Fujita M. .Matsumoto T. .et al Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Eur Respir J. 2013;41:243-245 [PubMed]journal. [CrossRef] [PubMed]
 
Ofek E. .Sato M. .Saito T. .et al Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol. 2013;26:350-356 [PubMed]journal. [CrossRef] [PubMed]
 
Reddy T.L. .Tominaga M. .Hansell D.M. .et al Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012;40:377-385 [PubMed]journal. [CrossRef] [PubMed]
 
Piciucchi S. .Tomassetti S. .Casoni G. .et al High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 2011;12:111- [PubMed]journal. [CrossRef] [PubMed]
 
Watanabe K. .Nagata N. .Kitasato Y. .et al Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Investig. 2012;50:88-97 [PubMed]journal. [CrossRef] [PubMed]
 
von der Thüsen J.H. . Pleuroparenchymal fibroelastosis: its pathological characteristics. Curr Respir Med Rev. 2013;9:238-247 [PubMed]journal. [PubMed]
 
Watanabe K. . Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev. 2013;9:- [PubMed]journal
 
Harada T. .Yoshida Y. .Kitasato Y. .et al The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis. Eur Respir Rev. 2014;23:263-266 [PubMed]journal. [PubMed]
 
Camus P. .von der Thüsen J. .Hansell D.M. .Colby T.V. . Pleuroparenchymal fibroelastosis: one more walk on the wild side of drugs? Eur Respir J. 2014;44:289-296 [PubMed]journal. [CrossRef] [PubMed]
 
Nakatani T. .Arai T. .Kitaichi M. .et al Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 2015;45:1183-1186 [PubMed]journal. [PubMed]
 
Chen F. .Matsubara K. .Miyagawa-Hayashino A. .et al Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy. Ann Thorac Surg. 2014;98:e115-e117 [PubMed]journal. [CrossRef] [PubMed]
 
Raghu G. .Collard H.R. .Egan J.J. . ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosiset al An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788-824 [PubMed]journal. [CrossRef] [PubMed]
 
Becker C.D. .Gil J. .Padilla M.L. . Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol. 2008;21:784-787 [PubMed]journal. [CrossRef] [PubMed]
 
Oda T. .Ogura T. .Kitamura H. .et al Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146:1248-1255 [PubMed]journal. [CrossRef] [PubMed]
 
Woodring J.H. .Barrett P.A. .Rehm S.R. .Nurenberg P. . Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. AJR Am J Roentgenol. 1989;152:743-747 [PubMed]journal. [CrossRef] [PubMed]
 
Beynat-Mouterde C. .Beltramo G. .Lezmi G. .et al Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J. 2014;44:523-527 [PubMed]journal. [CrossRef] [PubMed]
 
Enomoto N. .Kusagaya H. .Oyama Y. .et al Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF). BMC Pulm Med. 2014;14:91- [PubMed]journal. [CrossRef] [PubMed]
 
Hirota T. .Yoshida Y. .Kitasato Y. .et al Histological evolution of pleuroparenchymal fibroelastosis. Histopathology. 2015;66:545-554 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 Initial chest radiograph (A) demonstrates a small left apical pneumothorax and bilateral subpleural reticulation, most pronounced in the upper lobes. Initial noncontrast high-resolution computed tomography of the chest in the coronal plane (B), axial supine inspiratory view of the apices (C), and axial prone inspiratory view of the bases (D) demonstrates a small left hydropneumothorax and bilateral upper zone predominant irregular pleuroparenchymal thickening, architectural distortion, traction bronchiectasis, and tracheomegaly. There is relative sparing of the lower lobes, which demonstrate nonspecific subpleural reticulations, which can be seen in the setting of usual interstitial pneumonia or idiopathic pulmonary fibrosis. No evidence of honeycombing, bullous disease, or mosaic attenuation is identified.Grahic Jump Location
Figure Jump LinkFigure 2 Noncontrast high-resolution computed tomography of the chest in the coronal plane (A), axial supine inspiratory view of the apices (B), inferior upper lobes (C), and bases (D) at 5-month follow-up demonstrates new pneumomediastinum extending into the soft tissues of the neck and inferiorly to the level of the diaphragm as well as a new right apical pneumothorax and persistent left apical pneumothorax. There has been interval mild progression of the upper lobe predominant subpleural reticulation. Interlobular septal thickening is present, and the lung bases are unchanged from prior examination. E, Sagittal reformatted views demonstrate anteroposterior flattening of the chest wall.Grahic Jump Location
Figure Jump LinkFigure 3 Surgical lung biopsy with low-power (×2) magnification with hematoxylin and eosin stain depicting pleural (large arrows pointing to pleura) and subpleural fibrosis and elastosis (A). There is subpleural accentuation of fibroelastosis with an abrupt transition to normal lung tissue (small arrows), and higher-power (×10) magnification with elastic staining demonstrating fragmented elastic fibers and prominent subpleural alveolar septal elastosis obliterating the lung architecture (B). Vascular involvement was also present, with a small muscular artery with recanalized thrombus (short arrow) and medial hyperplasia (long arrow) seen (×10) (C), and another vessel with luminal obliteration and fragmented elastic fibers (×10) (D).Grahic Jump Location

Tables

References

Amitani R. .Miimi A. .Kuse F. . Idiopathic pulmonary upper lobe fibrosis (IPUF). Kokyu. 1992;11:693-699 [PubMed]journal
 
Frankel S.K. .Cool C.D. .Lynch D.A. .Brown K.K. . Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126:2007-2013 [PubMed]journal. [CrossRef] [PubMed]
 
Travis W.D. .Costabel U. .Hansell D.M. . ATS/ERS Committee on Idiopathic Interstitial Pneumoniaset 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;188:733-748 [PubMed]journal. [CrossRef] [PubMed]
 
Kusagaya H. .Nakamura Y. .Kono M. .et al Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 2012;12:72- [PubMed]journal. [CrossRef] [PubMed]
 
von der Thusen J.H. .Hansell D.M. .Tominaga M. .et al Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011;24:1633-1639 [PubMed]journal. [CrossRef] [PubMed]
 
Hirota T. .Fujita M. .Matsumoto T. .et al Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Eur Respir J. 2013;41:243-245 [PubMed]journal. [CrossRef] [PubMed]
 
Ofek E. .Sato M. .Saito T. .et al Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol. 2013;26:350-356 [PubMed]journal. [CrossRef] [PubMed]
 
Reddy T.L. .Tominaga M. .Hansell D.M. .et al Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012;40:377-385 [PubMed]journal. [CrossRef] [PubMed]
 
Piciucchi S. .Tomassetti S. .Casoni G. .et al High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 2011;12:111- [PubMed]journal. [CrossRef] [PubMed]
 
Watanabe K. .Nagata N. .Kitasato Y. .et al Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis. Respir Investig. 2012;50:88-97 [PubMed]journal. [CrossRef] [PubMed]
 
von der Thüsen J.H. . Pleuroparenchymal fibroelastosis: its pathological characteristics. Curr Respir Med Rev. 2013;9:238-247 [PubMed]journal. [PubMed]
 
Watanabe K. . Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev. 2013;9:- [PubMed]journal
 
Harada T. .Yoshida Y. .Kitasato Y. .et al The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis. Eur Respir Rev. 2014;23:263-266 [PubMed]journal. [PubMed]
 
Camus P. .von der Thüsen J. .Hansell D.M. .Colby T.V. . Pleuroparenchymal fibroelastosis: one more walk on the wild side of drugs? Eur Respir J. 2014;44:289-296 [PubMed]journal. [CrossRef] [PubMed]
 
Nakatani T. .Arai T. .Kitaichi M. .et al Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 2015;45:1183-1186 [PubMed]journal. [PubMed]
 
Chen F. .Matsubara K. .Miyagawa-Hayashino A. .et al Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy. Ann Thorac Surg. 2014;98:e115-e117 [PubMed]journal. [CrossRef] [PubMed]
 
Raghu G. .Collard H.R. .Egan J.J. . ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosiset al An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788-824 [PubMed]journal. [CrossRef] [PubMed]
 
Becker C.D. .Gil J. .Padilla M.L. . Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol. 2008;21:784-787 [PubMed]journal. [CrossRef] [PubMed]
 
Oda T. .Ogura T. .Kitamura H. .et al Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146:1248-1255 [PubMed]journal. [CrossRef] [PubMed]
 
Woodring J.H. .Barrett P.A. .Rehm S.R. .Nurenberg P. . Acquired tracheomegaly in adults as a complication of diffuse pulmonary fibrosis. AJR Am J Roentgenol. 1989;152:743-747 [PubMed]journal. [CrossRef] [PubMed]
 
Beynat-Mouterde C. .Beltramo G. .Lezmi G. .et al Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J. 2014;44:523-527 [PubMed]journal. [CrossRef] [PubMed]
 
Enomoto N. .Kusagaya H. .Oyama Y. .et al Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF). BMC Pulm Med. 2014;14:91- [PubMed]journal. [CrossRef] [PubMed]
 
Hirota T. .Yoshida Y. .Kitasato Y. .et al Histological evolution of pleuroparenchymal fibroelastosis. Histopathology. 2015;66:545-554 [PubMed]journal. [CrossRef] [PubMed]
 
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543