Intraductal papillary mucinous neoplasms (IPMNs) could increasingly be seen as a comorbidity in patients with pulmonary arterial hypertension (PAH), especially as their life expectancy increases. This is according to a new case study published in the Chest journal and being presented at the Chest 2021 Annual Meeting.

More research is needed to see whether the frequency of comorbidities increases as the life expectancy of PAH patients lengthens, according to the authors of the study. “As their life expectancy continues to lengthen, we expect to see other diseases with increasing frequency and will need a better understanding of the disease process to manage appropriately,” they said.

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The researchers reported for the first time a possible association between PAH and IPMN, 2 seemingly unrelated rare diseases. They described the case of 6 patients with PAH who were being treated at the University of Colorado Hospital with intravenous epoprostenol for an average of 14 years.

The patients presented with gastrointestinal symptoms such as abdominal pain, vomiting, diarrhea, and weight loss and upon investigation were diagnosed with IPMN. Because the patients were at high risk due to their PAH, they were not given a biopsy or treated surgically. Instead, they each received conservative symptom-driven support.

According to the authors, the identification of 6 patients with both diseases at a single center suggests that the 2 conditions could be sharing the same pathobiology. More studies based on the registry are necessary to establish the potential link between the 2 diseases, they said.

SMAD signaling has been linked to both pancreatic cancer and PAH. The researchers suggested that an imbalance in TGF-β/SMAD signaling could be triggering both vascular proliferation in PAH and the growth of IPMN.

IPMN and PAH are both rare diseases. The reported annual incidence of IPMN is less than 5 cases per 100,000 people. That of PAH is between 15 and 52 cases per 1 million people.

The Chest 2021 Annual Meeting is being held virtually Oct. 17-20, 2021.


Lee MH, Doran J, Bang TJ, et al. Pulmonary arterial hypertension and intraductal papillary mucinous neoplasms of the pancreas: a novel association? Chest. Published online October 11, 2021. doi:10.1016/j.chest.2021.06.078