In patients with sarcoidosis-associated pulmonary hypertension (PH), reduced diffusion capacity and 6-minute walk distance (6MWD) were associated with reduced transplant-free survival, according to study results published in the European Respiratory Journal.

An international registry of clinical data from patients with sarcoidosis-associated pulmonary (PH) known as ReSAPH was analyzed for potential associations that may be predictive of disease outcomes. Information analyzed included right heart catheterization data, pulmonary function testing, chest x-ray Scadding stage, and 6MWD.

Of the 215 patients with sarcoidosis-associated PH in the ReSAPH registry, 73.9% had precapillary PH and 60% were enrolled from the United States. Additionally, the mean age was 56.9 years and 72.3% of patients were women. Overall, the Kaplan-Meier adjusted 1, 3, and 5-year transplant free survival rates were 89.2%, 71.7% and 62.0%, respectively. While demographic characteristics were not predictive of outcomes, severe gas transfer impairment and 6MWD <300 m were strong predictors of decreased transplant-free survival.

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“Although this analysis has inherent limitations of registry data including some missing data, it clearly demonstrates that functional impairment (as demonstrated by the 6MWD) and reduced diffusing capacity are associated with decreased survival in [sarcoidosis-associated PH] patients,” the researchers wrote.


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Disclosure: Gilead Pharmaceuticals provided an unrestricted educational grant to support the registry. Please see the original reference for a full list of authors’ disclosures.

Reference

Shlobin OA, Kouranos V, Barnett SD, et al. Physiological predictors of survival in patients with sarcoidosis associated pulmonary hypertension: results from an international registry [published March 26, 2020]. Eur Respir J. doi: 10.1183/13993003.01747-2019