Abstract Introduction Patients with idiopathic pulmonary fibrosis (IPF) are at high risk of developing lung cancers given their increased risk of pulmonary metaplasia. The most common is squamous cell carcinoma (SCC), which is more aggressive in IPF, however, it may not be the direct cause of mortality. Here we present a patient with SCC and IPF that was complicated by right heart failure over only 4 months. Case A 62-year-old woman with presumed usual interstitial pneumonitis (UIP) interstitial lung disease (ILD) and stage III SCC presented to the emergency department with hypoxia to 80% and hypotension to 80/65. She had completed cycles of Carboplatin/Paclitaxel and was now undergoing radiation therapy. Her risk factors included 20 pack-years of tobacco smoking and 35 years of weekly crack cocaine. Serologic workup for connective tissue disease was negative, aside from a positive anti-nuclear antibody, so her ILD was attributed to IPF. CT chest showed progression of her ILD. Labs were notable for an elevated BNP in the 400s, when previously it had been normal. She was given intravenous diuretics and steroids without improvement to her oxygen requirements. A transthoracic echo revealed new flattened interventricular septum during systole, a severely dilated right ventricle, moderate right ventricle dysfunction, severe tricuspid regurgitation, and severe pulmonary hypertension that was not seen 4 months prior. 2 weeks into hospitalization, she continued to desaturate to 80% while on 70 liters of 100% FiO2 high-flow nasal cannula. Given her active cancer, she was deemed neither a candidate for solid organ transplantation nor extracorporeal membrane oxygenation. Since she continued to deteriorate clinically, the patient elected to pursue comfort care. Soon after, she expired from complications of progressive pulmonary fibrosis, pulmonary hypertension, and cardiogenic shock from her right heart failure. Discussion This case highlights the importance of early workup, diagnosis, and treatment for IPF and group III pulmonary arterial hypertension in patients with malignancy, as it can guide management. Radiation therapy to treat her malignancy should have been reconsidered, or at least, less frequent, given its potential to hasten pulmonary fibrosis and vascular remodeling. Early initiation of anti-fibrotic agents should be prioritized to improve longevity, as this patient survived less than 1 year from diagnosis of lung cancer and IPF. Lastly, signs of worsening PAH, right heart failure, and occult volume overload should be assessed in order to improve respiratory symptoms, regardless of long-term prognosis. This abstract is funded by: None
Sriparna et al. (2026) studied this question.