Virginia Via Research Day Book 2026

Medical Student Research Case Reports

33 IMPROVEMENT IN PULMONARY FUNCTION TESTING IN IDIOPATHIC PULMONARY FIBROSIS WITH COEXISTING PULMONARY HYPERTENSION FOLLOWING TREATMENT WITH INHALED TREPROSTINIL AND NINTEDANIB.

Connor Chapman, OMS-III; Robert Jones, MD Corresponding author: cchapman@vcom.edu VCOM-Virginia, Blacksburg, Virginia Ballad Health Medical Associates Pulmonology

04/2025, PFTs revealed a forced vital capacity (FVC) of 73% predicted (2.80L, DLCO 39%, DLVA 67%). HRCT showed moderate/severe pulmonary fibrosis with honeycombing and bronchiectasis. It also showed PH (verified by right heart catheterization - 07/2025). The patient was seen subsequently in 07/2025 (beginning Ofev 100 mg BID 4 weeks prior). At this visit, he was started on a Tyvaso inhaler (48 mcg). Of note, now he was only using oxygen at night (3L). Following this, he reported reduced dyspnea and increased exercise capacity. The patient had even begun strength training again. Repeat PFTs in 10/2025 demonstrated an FVC of 85% predicted (2.95L, DLCO 46%, DLVA 64%) representing a 12% increase. No significant adverse effects attributable to therapy were reported (other than diarrhea) and his symptoms greatly improved. There was no mention of supplemental oxygen. Comments: This case is notable for objective improvement in FVC in a patient with established IPF/PH after inhaled Treprostinil and Nintedanib. In recent literature, FVC has been considered the most reliable endpoint linked with patient outcome. The INCREASE and TETON studies have shown the FVC benefit of Tyvaso (FVC improvement of 1-8%, 28.5

mL at 8 weeks, and 44.4 mL at 16 weeks) (Nathan, et al.). Our patient showed a 12% increase in FVC (150 mL increase) at 12 weeks, significantly greater than what current literature shows. Although variability in spirometry and natural disease fluctuation should be considered, the magnitude and temporal association of the FVC increase with treatment, along with subjective/ symptomatic improvement, suggest a treatment related effect. This finding supports further investigation of combined antifibrotic and PH-targeted therapies in IPF/ PH as there is not much literature examining combined regimens. We report a patient who experienced meaningful improvement in FVC and symptoms following treatment with inhaled Treprostinil and Nintedanib. While causality cannot be established from one case, this observation is hypothesis-generating and highlights the importance of close monitoring and reporting of atypical positive trajectories in IPF /PH. Diagnosis: Idiopathic Pulmonary Fibrosis, Pulmonary Hypertension. IRB Statement: N/A.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease of the lungs characterized by a decline in lung function, dyspnea, and poor prognosis. Nintedanib (Ofev), a tyrosine kinase inhibitor, has been shown to slow disease progression by blocking growth factors (Richeldi, et al). When pulmonary hypertension (PH) is associated secondarily to IPF, like our patient, this complicates disease. Inhaled Treprostinil (Tyvaso), a prostacyclin analogue and vasodilator, has been shown to alleviate PH (Waxman, et al.). Notable improvements in pulmonary function testing (PFTs) in one with established IPF/ PH is uncommon even with these medications. In addition, not much literature exists on a combined Treprostinil/Nintedanib regimen, efficacy, and tolerance. In this case report, I describe a patient with IPF and PH who demonstrated a significant improvement in PFT parameters following inhaled Treprostinil and Nintedanib. Case Presentation: A 71-year-old male presented with progressive dyspnea and digital clubbing in 03/2025. 6-minute walk test prompted the need for 3L of oxygen to maintain >88%. The patient was advised to use 3L of oxygen for exertion and nighttime. In

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2026 Research Recognition Day