A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis

Our plain-English summary of A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis, by H. Huang, R. Feng, S. Li, et al (Medicine, 2017).

The study

• Authors: H. Huang, R. Feng, S. Li, B. Wu, K. Xu, Z. Xu, J. Chen
• Journal: Medicine
• Published: 2017;96(19):e6900
• Type of article: Case report
• Cited by: 9 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Rational: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE.

Patient Concerns: A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years. He had a "flattened thoracic cage" and bibasilar inspiratory crackles without finger clubbing. A series of chest computed tomography scans during the preceding 10 years revealed the presence of gradual, exaggerated, upper lung-predominant, diffuse pleural thickening and dense subpleural opacification with traction bronchiectasis.

Diagnosis: He was performed with video-assisted thoracic surgical (VATS) lung biopsy. The pulmonary histopathologic examination showed thickened visceral pleura and prominent subpleural fibroelastosis, confirming the diagnosis of iPPFE.

Intervention: After the failure of treatment with prednisone plus cyclophosphamide and sequential pirfenidone administration, he was arranged with bilateral lung transplantation two years later.

Outcomes: The patient did not require supplemental oxygenation anymore after he recovered from lung transplantation.

Lessons: Bilateral lung transplantation might be tried for the end-stage iPPFE cases.

In plain English

This was a case report from China about one man with idiopathic pleuroparenchymal fibroelastosis (iPPFE), a rare interstitial lung disease. The article states that he had no asbestos or cigarette exposure. He was 34 when admitted with cough and breathlessness on exertion that had worsened over 10 years. CT scans showed pleural thickening mainly in the upper lungs. A lung function test in November 2013 showed restricted breathing, with total lung capacity at 33.1% of predicted. A surgical lung biopsy in December 2013 confirmed iPPFE. Prednisone and cyclophosphamide were stopped after 8 months because his condition had not improved. About 6 months of pirfenidone, starting February 2015, was followed by further decline, with resting pulse oxygenation below 90%. In May 2016 he had a bilateral lung transplant. Afterwards he no longer needed supplemental oxygen, and the removed lungs confirmed PPFE. The authors concluded that bilateral lung transplantation might be tried for end-stage iPPFE. They noted that follow-up after the transplant had lasted only about 6 months.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 34-year-old man in China with idiopathic pleuroparenchymal fibroelastosis, treated at Peking Union Medical College Hospital, with transplantation in May 2016
• Exposure: The patient was reported to have no asbestos or cigarette exposure. The report concerns treatments: prednisone plus cyclophosphamide, then pirfenidone, then bilateral lung transplantation.
• Main results:
  • A lung function test in November 2013 showed restrictive dysfunction and impaired diffusion: FVC 1.3 L (41.2% of predicted), FEV1/FVC 84.7%, total lung capacity 2.31 L (33.1% of predicted), DLco 4.12 mmol/min/kPa (36.5% of predicted).
  • A video-assisted thoracic surgical lung biopsy in December 2013 showed thickened visceral pleura and prominent subpleural fibroelastosis, confirming iPPFE.
  • Prednisone and cyclophosphamide, started January 2014, were discontinued because the patient had not improved after 8 months.
  • After about 6 months of pirfenidone (1800 mg/d) from February 2015, the patient's respiratory condition gradually worsened and his resting pulse oxygenation was below 90%.
  • After bilateral lung transplantation in May 2016 and recovery, the patient did not require supplemental oxygen.
  • PPFE was confirmed by pathology on the lungs removed at transplantation.
• The authors' conclusion: The authors concluded that bilateral lung transplantation might be tried for end-stage iPPFE cases.
• Limitations the authors state:
  • The patient took pirfenidone for only about 6 months before transplantation.
  • Follow-up was only about 6 months after the transplant, and the authors said the patient should be followed longer.

Topics

Medical terms in this study, explained in our Lexicon: Lung Transplant, Idiopathic, Interstitial Lung Disease, Pleural Thickening, Fibrosis, Visceral Pleura, Dyspnea, Clubbing.

Related studies

• Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? — C.D. Becker, J. Gil, et al. (2008)
• Mesothelioma after lung transplantation — P.N. Chhajed, L. Bubendorf, et al. (2006)
• Asbestos exposure and tuberculous pleurisy as developmental causes of progressive unilateral upper-lung field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis — A. Sekine, E. Hagiwara, et al. (2021)
• Pleuroparenchymal Fibroelastosis With Long History of Asbestos and Silicon Exposure — L. Xu, N. Rassaei, et al. (2018)
• The comparison of high-resolution computed tomography findings in asbestosis and idiopathic pulmonary fibrosis — M. Akira, K. Morinaga (2016)
• Idiopathic pulmonary fibrosis in asbestos-exposed workers — E.A. Gaensler, P.J. Jederlinic, et al. (1991)

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