Multiple complications of advanced IPF in an individual patient presentation

Abstract

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