PH Grand Rounds: Confronting the Challenge of Sarcoidosis-Associated Pulmonary Hypertension

Pulmonary hypertension (PH) associated with sarcoidosis (World Health Organization Group 5) carries a poor prognosis and likely occurs through multiple mechanisms. Routine monitoring and a high clinical suspicion must be maintained to establish early diagnosis. Imaging and other ancillary tests may suggest PH in these patients, but as with all PH groups, right heart catheterization is needed for confirmation and for differentiating contributors to PH. Although there appears to be a role for treatment in select patients with significant concomitant pulmonary arterial hypertension (PAH), pulmonary vasodilator therapy can also risk worsening hypoxia secondary to ventilation/perfusion mismatch. The medical management of patients with sarcoidosis-associated pulmonary hypertension (SAPH) will be better informed with further study in large, randomized trials. At this time, there are no US Food and Drug Administration-approved therapies, including PAH medications, for patients with SAPH.