PURPOSE: Objective: Pulmonary arterial hypertension (PAH) is a known complication of sarcoidosis, however the incidence and outcome of sarcoidosis-associated pulmonary hypertension is not well known. More over, there are no randomized clinical trials addressing efficacy of available treatments for PAH in this sub group of patients. This study aims at describing the response to Bosentan therapy in four patients with sarcoidosis-associated pulmonary hypertension.
METHODS: Methods: Retrospective study of patients with sarcoidosis-associated pulmonary hypertensiontreated with Bosentan in a major urban teaching medical center.We identified 4 patients with chronic pulmonary sarcoidosis and clinical evidence of pulmonary arterial hypertension. After obtaining transthoracic echocardiogram, all patients underwent a right cardiac catheterization to confirm the diagnosis of PAH, and to obtain other important hemodynamic parameters. Baseline pulmonary function tests as well as a 6 minute walk test were (6MWT) obtained.All patients were started on Bosentan. They were monitored sequentially for drug side effects and their clinical response. A (6MWT) was repeated sequentially after initiating therapy with bosentan.
RESULTS: Results: There were 3 female patients and one male patient. The mean DLCO at baseline was 31.75 % of predicted. One patient was New York Heart Association (NHYA) functional class IV and was unable to do a 6 minute walk test. Among the other 3 patients, the NYHA functional class improved by one class after 6 months of Bosentan therapy. After therapy, 6MWT as well as oxygen desaturation improved in three patients by an average of 10%. The fourth patient, who could not perform the 6 MWT before therapy, reported subjective improvement. Repeated laboratory and clinical evaluations did not suggest any drug related adverse events in these patients.
CONCLUSION: Conclusion: Bosentan represents a valuable treatment option for patients with sarcoidosis-associated pulmonary hypertension. Overall, long-term treatment with Bosentan was well tolerated in all 4 cases.
CLINICAL IMPLICATIONS: In the future, a randomized controlled clinical trial will be warranted to further investigate the use of Bosentan in patients with PAH associated sarcoidosis.
DISCLOSURE: Christian Bimenyuy, No Financial Disclosure Information; No Product/Research Disclosure Information