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Updated: Aug 20, 2026

Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity
Stephen K Frankel1, Carlyne D Cool, David A Lynch
1National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA.
Abstract:
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.
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