Respiratory investigation · 2026
Early risk response to vasodilators and outcomes in interstitial lung disease associated pulmonary hypertension.
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Teramachi Ryo, Fukihara Jun, Ichihara Seiya, Kataoka Kensuke, Takei Reoto, Yamano Yasuhiko, Watanabe Fumiko, Yokoyama Toshiki, Kimura Tomoki, Kondoh Yasuhiro
Аннотация
BACKGROUND: Pulmonary hypertension (PH) commonly complicates interstitial lung disease (ILD) and is associated with poor prognosis. Although pulmonary vasodilators may improve clinical parameters, they can also cause severe hypoxemia; thus, reliable noninvasive tools are needed to assess treatment response. We investigated whether changes in four-stratum risk stratification category before and after vasodilator therapy are associated with prognosis in patients with ILD-PH.
METHODS: Clinical data before and after vasodilator therapy were retrospectively collected from consecutive patients with ILD-PH at a single ILD-specialty center in Japan between May 2007 to September 2023. Four-stratum risk stratification was performed using World Health Organization functional class, brain natriuretic peptide or N-terminal pro-brain natriuretic peptide, and 6-min walk distance. Cox proportional hazard analyses adjusted for ILD-gender-age-physiology index, mean pulmonary arterial pressure, and time from PH diagnosis to pulmonary vasodilator therapy were used to evaluate associations between risk stratification and transplant-free survival.
RESULTS: Among 64 patients, 6 were categorized as low risk, 30 as intermediate-low risk, 21 as intermediate-high risk, and 7 as high risk. Vasodilator therapy improved pulmonary hemodynamics and led to improvement in risk category in 9 patients and deterioration in 14. Post-treatment risk stratification provided good prognostic discrimination. Multivariate Cox analysis showed that post-treatment risk scores were independently associated with transplant-free survival (adjusted HR 3.286, 95% CI 1.897-6.258).
CONCLUSIONS: Post-treatment four-stratum risk stratification was associated with transplant-free survival in patients with ILD-PH.
METHODS: Clinical data before and after vasodilator therapy were retrospectively collected from consecutive patients with ILD-PH at a single ILD-specialty center in Japan between May 2007 to September 2023. Four-stratum risk stratification was performed using World Health Organization functional class, brain natriuretic peptide or N-terminal pro-brain natriuretic peptide, and 6-min walk distance. Cox proportional hazard analyses adjusted for ILD-gender-age-physiology index, mean pulmonary arterial pressure, and time from PH diagnosis to pulmonary vasodilator therapy were used to evaluate associations between risk stratification and transplant-free survival.
RESULTS: Among 64 patients, 6 were categorized as low risk, 30 as intermediate-low risk, 21 as intermediate-high risk, and 7 as high risk. Vasodilator therapy improved pulmonary hemodynamics and led to improvement in risk category in 9 patients and deterioration in 14. Post-treatment risk stratification provided good prognostic discrimination. Multivariate Cox analysis showed that post-treatment risk scores were independently associated with transplant-free survival (adjusted HR 3.286, 95% CI 1.897-6.258).
CONCLUSIONS: Post-treatment four-stratum risk stratification was associated with transplant-free survival in patients with ILD-PH.