Screening, diagnosis, and therapy of pulmonary hypertension (PH) in sarcoidosis patients with unexplained dyspnea is a common problem for pulmonary medicine clinicians. Sarcoidosis-associated pulmonary hypertension (SAPH) is most common in individuals with pulmonary fibrosis, but it can occur without any obstruction or limitation to airflow. SAPH is associated with dramatically increased mortality and lower functional ability, both of which can be avoided with early identification and targeted therapy. The pathophysiology of SAPH, which may mirror pulmonary arterial hypertension and secondary causes of PH, was discussed. Researchers provided a SAPH screening strategy and argued for a thorough examination of the etiology of PH in each patient before recommending a personalized treatment approach. They highlighted that immunosuppressive therapy for sarcoidosis was often insufficient to appropriately cure SAPH. They examined the secondary causes of SAPH, such as left heart disease, sleep apnea, and thromboembolic illness, as well as the evidence for using PH-specific medication in selected patients of SAPH. SAPH should be managed by PH-experienced doctors, including early referral to transplantation in refractory patients.
Reference:journals.lww.com/clinpulm/Abstract/2018/03000/Sarcoidosis_associated_Pulmonary_Hypertension_.4.aspx
