The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis

Harada, Taishi; Yoshida, Yūji; Kitasato, Yasuhiko; Tsuruta, Nobuko; Wakamatsu, Kentaro; Hirota, Takako; Tanaka, Makoto; Tashiro, Naoki; Ishii, Hiroshi; Shiraishi, Motokimi; Fujita, Masaki; Nagata, Nobuhiko; Watanabe, Kentaro

doi:10.1183/09059180.00006713

Cited by 70 publications

(61 citation statements)

References 6 publications

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“…Chest deformity (also referred to as platythorax) increased during the progression of PPFE [8] and this finding was also present in five of our own patients. In a review of all lung toxicity cases suspected of being caused by cyclophosphamide over a 20-year period, MALIK et al [9] identified two distinct patterns of cyclophosphamide-induced lung injury: 1) early onset pneumonitis that improved after cessation of the drug; and 2) late-onset pleuropulmonary fibrosis developing and progressing after cyclophosphamide was discontinued.…”

Section: Table 1 Continuedmentioning

confidence: 52%

Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

et al. 2014

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Section: Table 1 Continuedmentioning

confidence: 52%

Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

et al. 2014

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“…Kobayashi et al also reported the rapid decrease of FVC in a case of IPUF despite the steroid treat- (5). Although the use of FVC to assess the progression of IPPFE has not been validated with a large cohort or in clinical trials, we summarized the annual decline of FVC in patients with IPPFE in previous reports (3,6,(10)(11)(12) in Table 2. The data showed that the decline of FVC in the present case during treatment with pirfenidone could be small as compared to those in other cases (Table 2).…”

Section: Discussionmentioning

confidence: 99%

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

et al. 2016

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The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. Highresolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.

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“…To date, PPFE patients have been reported to present a low BMI with a flattened chest and a restrictive pulmonary impairment, which was observed in all our patients [6][7][8][9] . These results indicate that one of the differences between our patients and patients with PPFE was whether a lesion existed in the unilateral lung or bilateral lungs.…”

Section: Discussionmentioning

confidence: 96%

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

Sekine

Satoh²,

Iwasawa³

et al. 2017

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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.

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The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis

Cited by 70 publications

References 6 publications

Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

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

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