Sarcoidosis-Associated Pulmonary Hypertension

Sarcoidosis is a multisystem disease characterized by granulomatous tissue infiltration. It predominantly affects the lung in most patients. Sarcoidosis-associated pulmonary hypertension (SAPH) is a common complication of advanced structural lung disease due to sarcoidosis but can also be observed as an isolated vascular phenomenon in patients with little to no structural lung disease. SAPH is associated with high mortality. The spectrum of pulmonary vascular involvement that causes SAPH appears to be quite variable. Furthermore, the optimal approach to treatment remains unclear. Various clinical trials investigating the use of PAH therapies to treat SAPH have yielded mixed results. Improvements in hemodynamics have generally been more clearly demonstrated as compared to other functional and symptomatic assessments. Unfortunately, there is very little controlled, randomized, prospective data in this regard. This chapter will explore what is known with respect to the various etiologies of SAPH, the clinical course and outcomes, and published data on the use of PAH therapies for SAPH.