Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy

Abstract

Background: Pleuroparenchymal fibroelastosis (PPFE) is a rare bilateral idiopathic interstitial pneumonia defined by pleural-parenchymal involvement. In clinical practice, we encountered patients with upper lung field pulmonary fibrosis (Upper-PF), which was radiologically consistent with PPFE, but apparently limited to the unilateral lung. Objectives: The purpose of the study was to clarify the clinical characteristics in those patients. Methods: We examined the medical records of all the consecutive patients from 2012 to 2016 to see whether there were patients having unilateral Upper-PF. Results: We found 6 patients with unilateral Upper-PF. The most common symptom was dyspnea, and all patients had a low body mass index and severe restrictive pulmonary impairment. Notably, all patients had a history of thoracotomy for resecting lung or esophageal cancer, and the lesions were limited to the operated side. Dynamic breathing chest MRI showed an impaired thoracic movement in the operated side. Serial chest CT from prethoracotomy to the first visit was obtained in 5 patients: before thoracotomy, only a slight apical cap, defined as a wedge- and triangle-shaped opacity with broad pleural contact, was observed only in the operated side, but progressed into the lesion after a median of 8.4 years following thoracotomy. After the first visit, the unilateral lesion rapidly deteriorated in all patients. Conclusions: Unilateral Upper-PF had some characteristics in common with PPFE. Because the lesion was limited to the operated side, unilateral Upper-PF would be a new disease entity related to thoracotomy. Our results indicate that thoracotomy impairs thoracic movement in the operated side and subsequently triggers unilateral Upper-PF development, especially in patients with an apical cap.

Podoplanin‐positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis

“…4,14 The present study emphasised this difficulty further, even using immunohistochemistry. Interestingly, Sekine et al reported recently that lobectomy for lungs with PAC might be a risk for the development of PPFE, 15 suggesting the potential of PAC as a pre-PPFE lesion. Our and their observations may indicate a common pathogenesis, possibly including MMT, between PPFE and PAC, although it remains unclear what factors make the fibroelastosis lesions progressive.…”

Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia: A case report

“…PPFE might be related to relevant underlying conditions and coexist with other ILDs with lower lung involvement. As in the present case, PPFE can exist unilaterally[ 17 ]. In the near future, PPFE must be investigated further, and clinicians need more information, including the pathogenesis, etiology, prognosis and in particular an effective treatment for PPFE.…”

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis

“…Bone marrow, stem cell and lung transplantation can induce PPFE as a manifestation of graft versus host disease [7][8][9][10]. Occupational dust exposure such as asbestosis or aluminum may also elicit pleuroparenchymal fibroelastosis [11,12]. Mycobacterial disease or aspergillus infection have also been reported to precipitate PPFE [13][14][15].…”

“…The diagnostic probability of PPFE was designated as inconsistent, low, intermediate and definitive (Table 2) according to the existence of clinical, laboratory and radiologic manifestations of the disease. The proposed diagnostic assessment scoring profile was constituted according to the PPFE manifestations revealed in literature [2][3][4][5][6]12,14] and of our patient itself. Diagnostic assessment scoring scale revealed significant correlation with the clinical findings of the presented case that pointed out to a definitive and a final diagnosis of PPFE.…”