Chest
Volume 126, Issue 6, December 2004, Pages 2007-2013
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Selected Reports
Idiopathic Pleuroparenchymal Fibroelastosis: Description of a Novel Clinicopathologic Entity

https://doi.org/10.1378/chest.126.6.2007Get rights and content

Between 1996 and 2001, we identified five cases of a unique idiopathic pleuroparenchymal lung disease characterized by a clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, marked pleural and parenchymal radiographic involvement with an upper lobe predominance, and surgical lung biopsy findings that did not fit with any of the currently defined interstitial pneumonias. The pathologic findings included the following: (1) intense fibrosis of the visceral pleura; (2) prominent, homogenous, subpleural fibroelastosis; (3) sparing of the parenchyma distant from the pleura; (4) mild, patchy lymphoplasmacytic infiltrates; and (5) small numbers of fibroblastic foci present at the leading edge of the fibrosis. In this report, we characterize the clinical, radiographic, physiologic, and pathologic findings of this entity, which we term idiopathic pleuroparenchymal fibroelastosis.

Section snippets

Materials and Methods

The clinical database of the Interstitial Lung Disease Program at National Jewish Medical and Research Center was reviewed for cases of combined pleural and lung parenchymal fibrosis for visit dates between 1996 and 2001. All living study subjects had been prospectively enrolled in our specialized center of research longitudinal study of interstitial lung disease that was approved by our institutional review board and supported by the National Institutes of Health. Case records were abstracted

Clinical Features

Patient 1 was a 65-year-old woman with “incidental” pleural thickening seen on a chest radiograph 10 years prior to referral. Four years prior to referral, she underwent a modified radical mastectomy for adenocarcinoma of the breast and was treated with adjuvant chemotherapy of cyclophosphamide, methotrexate, and fluorouracil (CMF) without radiation therapy. The patient was treated with daily doses of tamoxifen, and she returned to her baseline function of aerobic exercise 60 min daily. Eight

Discussion

We report herein on five cases of a rare clinicopathologic syndrome with clinical features that overlap those of a chronic IIP. The most characteristic features of this disorder are the radiographic evidence of mid-lung zone and upper lung zone pleural and parenchymal abnormalities, and distinctive histopathologic findings on surgical lung biopsy specimens. In all five cases, the pathology was identical and was characterized by a combination of pleural and parenchymal fibroelastosis.

This

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