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Updated: Aug 2, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Acute exacerbation of pleuroparenchymal fibroelastosis with lower lobe usual interstitial pneumonia: An autopsy case
Takuma Matsumura1, Takashi Makino2, Marie Yagisawa1
1Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan.
Abstract:
An 87-year-old man presented with dyspnea. Computed tomography revealed progressive subpleural consolidation in the apex, reticular shadows in the lower lobes, and bilateral ground glass opacifications. He died of respiratory failure on day 3. The post-mortem examination showed exudative stage diffuse alveolar damage and pulmonary edema. Intraalveolar collagenous fibrosis and subpleural elastosis were observed in the upper lobes, accompanied by interlobular septal and pleural thickening and lung architecture remodeling in the lower lobes. He was diagnosed with acute exacerbation of pleuroparenchymal fibroelastosis with lower lobe usual interstitial pneumonia, which can be fatal.
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