Introduction and Objectives Pulmonary hypertension (PH) is increasingly recognised as a major contributor to poorer outcomes in pulmonary sarcoidosis. Current treatment guidelines for sarcoidosis associated pulmonary hypertension (SAPH) recommend the optimisation of underlying lung disease and use of oxygen when hypoxaemia is present,1 however there is insufficient evidence to recommend advanced therapies. Limited data suggest a benefit following treatment with sildenafil in SAPH, although no randomised controlled treatment trials have been reported. We report our experience of patients with SAPH treated with sildenafil.
Methods We reviewed 22 patients (mean age 56±12 years; 10 men) with SAPH (mean pulmonary artery pressure 46±9 mm Hg) who received treatment with sildenafil. Haemodynamic measurements were evaluated by right heart catheterisation in all patients. Serial measurements of brain natriuretic peptide (BNP), pulmonary function testing and functional status were collected.
Results The mean duration of follow-up after the commencement of sildenafil was 21±14 months. Six patients died during follow-up, and four patients required the addition of an endothelin receptor antagonist due to worsening pulmonary hypertension. Patients were dichotomised into responders (those maintained on sildenafil monotherapy; n=12) or non-responders (those who died or required and escalation of PH therapy during follow-up; n=10). Compared to responders, non-responders had a significantly lower % predicted DLco (20% vs 34%; p=0.03) and % predicted Kco (46% vs 62%; p=0.05) at commencement of sildenafil (Abstract P4 table 1). Within 6 months of commencement of sildenafil, non-responders had a 21% increase in median BNP levels, while responders had a 51% reduction in BNP levels. Sildenafil was ceased in one patient after the development of ocular side-effects attributed to the drug.
Conclusions Our observations suggest that sildenafil is safe and well tolerated in SAPH. At commencement of sildenafil, a lower % predicted DLco and Kco, and an increasing BNP level within the first 6 months of therapy, was associated with increased risk of death or requirement for additional PH therapy. Controlled trials are warranted before therapeutic recommendations can be made.
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