RT Journal Article
SR Electronic
T1 Outcomes from use of targeted therapy in pulmonary hypertension associated with sarcoidosis (PHAS)
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P948
VO 40
IS Suppl 56
A1 John Cannon
A1 Colm McCabe
A1 Joanna Pepke-Zaba
A1 Karen Sheares
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P948.abstract
AB Pulmonary hypertension is a recognised complication of sarcoidosis and may arise from several aetiological pathways. We report our experience of patients with PHAS-GroupV on targeted treatment.Retrospective study of patient outcomes with PHAS. All patients underwent right heart catheterisation satisfying criteria for diagnosis of PAH. We reviewed 16 patients, 10 of whom died in mean f/u 32m. Patients dichotomised into 2 groups (responders and non-responders) based on improvement in log ntproBNP and 6MWD within 6 months of therapy initiation.View this table:Treatment response was associated with a reduced risk of death. Cohort phenotypes suggest that treatment response may not be predicted from sarcoid stage, functional class, FVC or presence of extrapulmonary features. However elevation in PVR may possibly be associated with a favourable response. An RCT is indicated to evaluate a larger cohort.