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Case Reports
. 2023 Mar 24;11(4):e01118.
doi: 10.1002/rcr2.1118. eCollection 2023 Apr.

Multiple complications of advanced IPF in an individual patient presentation

Thomas Ledger  1 , Stanley Braude  1
Affiliations
Case Reports

Multiple complications of advanced IPF in an individual patient presentation

Thomas Ledger et al. Respirol Case Rep. .

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.

Keywords: acute exacerbation of idiopathic pulmonary fibrosis; idiopathic pulmonary fibrosis; platypnoea‐orthodeoxia syndrome; pneumomediastinum.

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Conflict of interest statement

None declared.

Figures

FIGURE 1
FIGURE 1
Ground glass changes present on a background of UIP pattern changes (traction bronchiectasis, subpleural cysts), showing changes in transverse and coronal planes before (A, B) and after treatment (C, D). The pneumomediastinum is visible on the lower images (C, D) as air surrounding the mediastinal structures.
See this image and copyright information in PMC

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