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Updated: Aug 5, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Multiple complications of advanced IPF in an individual patient presentation
Thomas Ledger1, Stanley Braude1
1Department of Respiratory Medicine Northern Beaches Hospital Sydney New South Wales Australia.
Abstract:
We report a patient with advanced idiopathic pulmonary fibrosis (IPF), who in a single presentation experienced three complications of the disease: an acute exacerbation, spontaneous pneumomediastinum, and platypnoea-orthodeoxia syndrome. Despite there being no definitive evidence-based treatment for an acute exacerbation, we report a marked improvement with high-dose steroids. This case also highlights the importance in IPF patients of considering pneumomediastinum as a cause of non-cardiac chest pain, as well as platypnoea-orthodeoxia in those with positional dyspnoea.
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