Backgrounds: Pulmonary sarcoidosis is often complicated by pulmonary hypertension, a complication that is associated with increased disability and mortality. To this point, however, little progress has been made in the treatment of sarcoidosis associated with pulmonary hypertension (SAPH).
Methods: A prospective study was performed on 72 consecutive Chinese sarcoidosis patients followed at an outpatient clinic. The patients were evaluated by Doppler echocardiography and computed tomography pulmonary angiography. SAPH was confirmed by right heart catheterisation. The clinical parameters were compared before and 2 months after treatment with oral glucocorticoids. Eight stage III and IV patients with moderate to severe proximal pulmonary arterial stenosis (PAS) and SAPH underwent interventional therapy (IT) after prednisone treatment and were followed up at 3-month intervals.
Results: After 2 months of prednisone treatment, 32 stage III and IV patients continued to display varying degrees of PAS and SAPH. Eight patients underwent IT without severe complications and made improvements in pulmonary arterial pressure, pulmonary vascular resistance, arterial oxygen saturation and WHO functional classification, with the improvements lasting more than 3 months.
Conclusions: PAS caused by external compression in sarcoidosis is a significant reason for SAPH. IT is effective and safe in the treatment of PAS and SAPH.
Keywords: interventional therapy; pulmonary arterial stenosis; pulmonary hypertension; sarcoidosis.
© 2016 John Wiley & Sons Ltd.