Outcomes of directed pah therapy in sarcoidosis.
Albujoq K, Awdish RL, Jennings JH, and Hegab S. Outcomes of directed pah therapy in sarcoidosis. Am J Respir Crit Care Med 2018; 197.
Am J Respir Crit Care Med
Pulmonary hypertension can develop as a complication of sarcoidosis, even in the absence of parenchymal involvement. Untreated sarcoidosis associated pulmonary hypertension (SAPH) is associated with poor prognosis and high mortality. While pulmonary transplant is the definitive treatment, not all patients are candidates for transplant. With the advent of advanced pulmonary hypertension therapies, there is potential to improve quality of life and functional status in patients who carry both diagnoses. The optimal management strategy for SAPH is unknown. Treatment has typically involved use of systemic anti-inflammatory medications, anticoagulants, pulmonary vasodilators, and supplemental oxygen. Use of pulmonary vasodilators in patients with pulmonary fibrosis has been limited, given concerns for severe hypoxemia or pulmonary edema caused by intrapulmonary shunting. Since SAPH is often caused by fibrosis with end-stage pulmonary microcirculatory “fixed” abnormalities it has also been unclear whether pulmonary vasodilators have any beneficial impact on the pulmonary vascular resistance. The available literature regarding SAPH therapeutic strategies is limited to small cohort studies, retrospective analyses, and case reports. Our study aimed to evaluate the subset of patients with sarcoidosis associated pulmonary hypertension and their response to pulmonary vasodilator therapies. Methods: The pulmonary hypertension database at Henry Ford Hospital was used to identify patients with a diagnosis of sarcoid who were treated with pulmonary vasodilators (including phosphodiesterase inhibitors, endothelin inhibitors, and prostacyclin analogues). Patients were included if they had 2D echocardiograms before and after treatment initiation. Exclusion criteria was known group 1 pulmonary hypertension. Paired hemodynamic endpoints were compared with t-test or Wilcoxon signed-rank test depending on normality of the data. Results: 50 patients with a diagnosis of SAPH were identified. Of these, 22 received treatment with pulmonary vasodilators. Patients with dual diagnoses who were treated had a statistically significant response to therapy for the following endpoints: PAP on 2D echo, 6MWD, and post therapy BNP. The median days between pre and post echocardiograms was 334 (IQR 140-499). PAP was significantly reduced following pulmonary vasodilators (77.7 vs 51.3 mm Hg, p <0.001) and brain natriuretic peptide (BNP) levels were lower (70 vs 94 ng/mL, p - 0.02). 6MWD was increased following therapy (276.1 vs 329.1 meters, p=0.006). Conclusion: In this preliminary study, treatment with pulmonary vasodilators resulted in a significant decrease in mean PAP, BNP, and an increase in 6MWD in patients with sarcoidosis and pulmonary hypertension. Further research is needed to establish the role of therapy in this subset of patients.