Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?

Our plain-English summary of Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?, by C.D. Becker, J. Gil, M.L. Padilla (Modern Pathology, 2008).

The study

• Authors: C.D. Becker, J. Gil, M.L. Padilla
• Journal: Modern Pathology
• Published: 2008;21(6):784-7
• Type of article: Case report
• Cited by: 94 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Idiopathic pleuroparenchymal fibroelastosis is a rare recently described entity likely to be under- and misdiagnosed, as awareness of this entity is not yet widespread. We report two cases that show the need to include this disease in the differential diagnosis of patients with predominantly pleural and subpleural fibrotic processes. The condition is a fibrotic thickening of the pleura and subpleural parenchyma due to elastic fiber proliferation predominantly in the upper lobes. Performing elastic fiber stains routinely in patients with fibrosis of this distribution may, therefore, aid in establishing the diagnosis and differentiating it from usual interstitial pneumonia/idiopathic pulmonary fibrosis. These patients may be prone to the development of secondary spontaneous pneumothoraces and persistent postoperative bronchopleural fistulae. Continued study of newly diagnosed cases may uncover shared characteristics or features helpful in generating an etiologic hypothesis. Only with better understanding of this disease can we hope in the future to be able to offer treatments other than supportive care and ultimately lung transplantation, which are the only therapeutic options available today.

In plain English

This article reported two cases of idiopathic pleuroparenchymal fibroelastosis. It is a rare disease in which elastic fibers thicken the pleura (the lung lining) and the tissue just beneath it, mainly in the upper lobes. Both patients were women, aged 51 and 59. The first reported no toxic exposures. The second, a librarian, reported possible asbestos exposure in childhood. Her lung tissue was first read as usual interstitial pneumonia/idiopathic pulmonary fibrosis, with no evidence of asbestos fibers; elastic fiber stains were not done. When her slides were reviewed, the diagnosis was changed to idiopathic pleuroparenchymal fibroelastosis. Both patients had a pneumothorax (air around the lung) and a bronchopleural fistula (a persistent air leak). The first patient's resolved after surgery. The second patient's persisted despite repeated operations; she died after septic complications. The authors listed asbestosis among diseases to consider when both pleura and lung tissue are scarred. They concluded that the condition is likely underdiagnosed and may be mistaken for idiopathic pulmonary fibrosis. They also concluded that routine elastic fiber stains in mainly pleural and subpleural fibrosis may help establish the diagnosis.

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 (two cases) with discussion
• Who was studied: Two women, aged 51 and 59, reported by physicians of the Mount Sinai School of Medicine, New York, USA. The second patient had her first surgery at another hospital and was then transferred to the authors' hospital.
• Exposure: Case 1 reported no ergot alkaloid use, illicit drug use or toxic exposures. Case 2, employed as a librarian, reported possible asbestos exposure in childhood and had past chemotherapy for follicular center cell lymphoma. No fiber type was named. The article did not study asbestos exposure as a cause.
• Main results:
  • Case 1: video-assisted thoracoscopy with wedge resections was complicated by a persistent right-sided pneumothorax with bronchopleural fistula, which resolved after the operation. The slides were diagnosed as idiopathic pleuroparenchymal fibroelastosis.
  • Case 2: the lung specimen was first interpreted as consistent with usual interstitial pneumonia/idiopathic pulmonary fibrosis, without any evidence of asbestos fibers. Elastic fiber stains were not performed.
  • Case 2: a large bronchopleural fistula persisted despite multiple operative revisions. The patient developed septic complications and died. On review of the outside slides, the diagnosis was amended to idiopathic pleuroparenchymal fibroelastosis.
  • Both patients developed pneumothorax and bronchopleural fistula complications. In case 1 this was a procedural complication. In case 2 it began as a secondary spontaneous pneumothorax.
  • Tissue in case 1 showed an abundance of short, curled, randomly oriented elastic fibers. The authors' cases did not display the honeycombing characteristic of usual interstitial pneumonia/idiopathic pulmonary fibrosis.
  • The authors listed asbestosis among the differential diagnoses for combined pleural and parenchymal fibrosis, together with connective tissue diseases, advanced fibrosing sarcoidosis, and radiation- or drug-induced lung disease.
• The authors' conclusion: The authors concluded that idiopathic pleuroparenchymal fibroelastosis is likely underdiagnosed and may be misdiagnosed as usual interstitial pneumonia/idiopathic pulmonary fibrosis. They concluded that routine elastic fiber stains in cases of predominantly pleural and subpleural fibrosis may aid diagnosis, and that its cause is unknown. They stated there is currently no effective treatment beyond lung transplantation.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Pleural plaques and thickening.

Medical terms in this study, explained in our Lexicon: Idiopathic, Differential Diagnosis, Pleura, Parenchyma, Fibrosis, Interstitial, Pulmonary Fibrosis, Lung Transplant.

Related studies

• A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis — H. Huang, R. Feng, et al. (2017)
• 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)
• Interstitial pulmonary fibrosis environmental or idiopathic? — G.L. Snider (1985)
• Relative release of interleukin-1 beta and interleukin-1 receptor antagonist by alveolar macrophages. A study in asbestos-induced lung disease, sarcoidosis, and idiopathic pulmonary fibrosis — J.N. Kline, D.A. Schwartz, et al. (1993)
• Asbestosis: demonstration of distinctive interstitial fibroelastosis: a pilot study — M.R. Wick, T.J. Kendall, et al. (2009)

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