A 56-Year-Old Woman With Worsening Dyspnea and Sarcoidosis

Kennedy O. Omonuwa, MD; Arunabh Talwar, MD; Lindsay Goodman, MD


October 31, 2019

Treatment of SAPH can be divided into primary therapy and advanced therapy. Primary therapy for SAPH consists of treating the underlying lung disease with, most commonly, corticosteroids and other immunosuppressive agents. Corticosteroid therapy has been shown to improve pulmonary hypertension in some patients with sarcoidosis, even in the absence of parenchymal fibrosis.

Advanced therapy is directed at the pulmonary hypertension and includes administration of antiproliferative pharmacologic agents that promote vasodilation. These agents include endothelin receptor antagonists (eg, bosentan), phosphodiesterase-5 inhibitors (eg, sildenafil), and prostanoids (eg, epoprostenol).

Lung transplantation should be considered in patients who have progressive or severe SAPH, particularly if the patient does not improve after initiation of advanced therapy.[1,4]

Pulmonary hypertension is a predictor of poor outcome in sarcoidosis; hence, mortality increases as the severity of the pulmonary hypertension increases. Physicians should have a high index of suspicion for this complication and should consider echocardiographic screening for pulmonary hypertension in the evaluation of persistent or unexplained dyspnea in patients with pulmonary sarcoidosis or other interstitial lung disease known to cause pulmonary hypertension, especially if the ECG shows findings suggestive of right-heart dysfunction. Those patients with elevated pulmonary artery systolic pressure on echocardiography should then be considered for right-heart catheterization as a means to establish the diagnosis and severity of pulmonary hypertension, and to assess the response to vasodilator therapy. Early identification of pulmonary hypertension in patients with pulmonary sarcoidosis may identify a subgroup of patients who require more aggressive vasodilator therapy rather than standard management of pulmonary hypertension.[1,2,4]

The patient in this case was admitted and evaluated by a pulmonary specialist and had right-heart catheterization done, which confirmed the diagnosis of pulmonary hypertension. She was started on bosentan and sildenafil for her pulmonary hypertension and continued to receive oral prednisone for her underlying pulmonary sarcoidosis. She was discharged the next day to follow up with a pulmonary hypertension clinic. After several months of outpatient therapy and monitoring, the patient's dyspnea and exercise tolerance considerably improved.


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