Sarcoidosis is a multisystem disease of unknown etiology that is characterized pathologically by noncaseating granulomatous inflammation with a particular predilection for the lungs. Its clinical course is highly variable, with a sizable proportion of patients experiencing spontaneous regression, while in others chronic disease develops.1
Pulmonary hypertension (PH) is a hemodynamic state defined as a mean pulmonary artery pressure of > 25 mm Hg at rest or > 30 mm Hg with exercise. PH is seen in a variety of clinical settings, notably left-sided valvular and myocardial disease, chronic lung disease with hypoxemia, and chronic pulmonary embolism (Table 1).2 PH with a pulmonary capillary wedge pressure of < 15 mm Hg is called pulmonary arterial hypertension (PAH), and is seen in association with connective tissue disorders, congenital heart disease, and HIV infection, among other conditions. When no clear etiology can be identified in patients with PAH, the disease is classified as idiopathic PAH (IPAH).2,3 While IPAH is less common than other forms of PH, it is the best studied mainly because it is a well-defined subset of the disease.3,4 Sarcoidosis-associated PH is one of the harder to define PH subsets and falls into the “miscellaneous” category in the current classification, highlighting its heterogeneity and our lack of complete understanding of this condition.