Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Abstract

Background: Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined. Methods: We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed. Results: Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n ‫؍‬ 18) increased by 59 m (p ‫؍‬ 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 ؎ 4.3 to 39.4 ؎ 2.8 mm Hg (p ‫؍‬ 0.008). The 1-and 3-year transplant-free survival rates were 90% and 74%, respectively. Conclusions: PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

“…After a median of 4 months of therapy, repeat haemodynamics and 6MWD were measured. There was a significant improvement in mean pulmonary artery pressure and pulmonary vascular resistance, similar to the data reported in prior studies [15][16][17][18]. Interestingly, there was no significant change in 6MWD.…”

“…An improvement in haemodynamics with little change in 6MWD has been noted in two prospective clinical trials that evaluated S-APH patients after 4 months of therapy [15,16]. Another study did report that some patients had improvement in their 6MWD after a median of 14 months treatment [17]. That study found that only those patients with none to moderate impairment in vital capacity had an improvement in their 6MWD.…”

Sarcoidosis Presenting with Inguinal Lympadenopathy and Treated with a Combination of Corticosteroids and Aazothioprine

“…[1] For the remaining patients, corticosteroids alone are the first choice (10-20 mg a day), but if corticosteroidsparing agents are necessary, then methotrexate or azothioprine are the best choices. [7] In our case, the patient was diabetic. Since higher steroid doses can impair glucose levels, we preferred to begin the treatment with lower doses of corticosteroids the day after protocol.…”

Prostacyclin-analog therapy in sickle cell pulmonary hypertension

“…There is precedence for similar reports. [10][11][12] To date, prostacyclin-analog therapy has not been recommended for SCDPH due to apparent fear of high-cardiac output pulmonary edema, line sepsis, and line thrombosis. The only relevant publication is a brief report describing tolerance of continuous intravenous epoprostenol in one patient for 1.5 years.…”