A39-16 Prostacyclin Therapy in Sarcoidosis-Associated Pulmonary Hypertension: A Single Health Care System Experience

Abstract Introduction Sarcoidosis-associated pulmonary hypertension (SAPH) is classified as WHO Group 5 pulmonary hypertension (PH) and shares pathophysiologic mechanisms with other PH groups, including vascular remodeling, fibrotic destruction of pulmonary parenchyma and hypoxic vasoconstriction. While evidence remains limited, prostacyclin therapy is considered in patients with precapillary SAPH who remain symptomatic despite optimal sarcoidosis management. This study is aimed to retrospectively review the outcomes of SAPH treated with prostacyclins in our system over the past 15 years. Methods A computerized database search was conducted in our electronic medical record to include the diagnostic code for “sarcoidosis (D86)” and medication “prostacyclin” from January 2010 to September 2025. Included subjects met either high probable likelihood on the WASOG Sarcoidosis Organ Assessment Instrument or demonstrated histologic evidence of granulomatous inflammation in the absence of alternative causes. Analysis of the data was done using descriptive statistics. Results 11 patients were diagnosed with SAPH in the study period. 7 patients were on prednisone, 1 was on mycophenolate mofetil and 3 patients were not on any immunosuppressants. The mean pulmonary artery pressure (mPAP) on the RHC ranged from 31 to 59 with an average mPAP of 38.3 mm Hg. At the time of diagnosis, all patients were ERS intermediate risk except patient 3 who was low risk. All patients except 3 were on concomitant phosphodiesterase 5 inhibitors and half the patients were on concomitant endothelin receptor agonists. 8 patients were on inhaled prostacyclin, 1 was on oral prostacyclin, 1 was on IV prostacyclin and 1 patient was on both inhaled and oral therapy. REVEAL LITE scores were calculated at 3 months except for 1 patient who was lost to follow up. At 3 months, 6 patients remained intermediate risk, 1 intermediate risk patient converted to low risk and 3 patients went up the risk class. At 1 year, 3 patients had passed away and 1 lost to follow up. For the remaining 7, 3 patients remained intermediate risk, 1 remained high risk, 2 patients stepped down in their risk class and 1 patient became a high risk from a low risk. Conclusion Our study suggested that SAPH is associated with a poor prognosis. Patients that are ERS intermediate risk at the time of diagnosis stabilized their risk class at 1 year interval when on triple PAH therapy with prostacyclins. An individualized treatment approach remains essential given the heterogeneous mechanisms of SAPH and limited trial data. This abstract is funded by: none