What is the epidemiology of clinically significant pulmonary hypertension in patients with sarcoidosis?

Bottom line:  Pulmonary hypertension can affect patients at any stage of sarcoid disease, but is most prevalent at more advanced stages.  Patients with sarcodosis who present with dyspnea on exertion, cough, chest pain or palpitations could have pulmonary hypertension, which is associated with a poorer prognosis, but these symptoms can also be associated with the sarcoidosis alone or other conditions.

Summary: Cordova FC, et al. Sarcoidosis-associated pulmonary hypertension. Curr Opin Pulm Med. 2013 Sep;19(5):531-7. doi: 10.1097/MCP.0b013e328363f4a3.

Shino MY, et al. Sarcoidosis-associated pulmonary hypertension and lung transplantation for sarcoidosis. Semin Respir Crit Care Med. 2014 Jun;35(3):362-71. doi: 10.1055/s-0034-1376863.
According to these recent reviews, PH occurs in 5-79% of patients with higher rates among patients with “advanced fribrocystic disease.”  The incidence of PH also varies according to the definition of PH.

In looking at clinical presentation of patients with sarcoidosis and PH, one study of 106 patients found no difference between sarcoidosis patients with and without PH in frequency of presentation with dyspnea on exertion, cough, palpitations, or chest pain. Radiographic findings were not significantly different.  (Chest. 2005 Sep;128(3):1483-9. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Sulica R, et al.)

Both Cordova and Shinu reference studies finding a statistically significant difference in the six minute walk test between patients with and without PH.

Pulmonary hypertension is associated with a poorer prognosis.  Shino and colleagues cite a retrospective study of 404 patients with sarcoidosis awaiting lung transplant in the US.   Three factors for increased risk of mortality included presence of PH, the amount of supplemental oxygen needed, and African American race.  (See Prognosis of Pulmonary Hypertension section.)


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Clinical Informationist at EUH Branch Library
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