Sarcoidosis-associated Pulmonary Hypertension Is Linked to a Poor Prognosis

Pulmonary hypertension in patients with sarcoidosis is associated with poor prognosis, despite treatment with a targeted therapy, a new study shows.

The study “Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension,” was published in the European Respiratory Journal.

Patients with sarcoidosis frequently develop pulmonary hypertension. In particular, symptomatic patients or those on the list for lung transplantation have a reported prevalence of 5 to 74 percent.

Pulmonary hypertension is a significant complication that can have major functional and prognostic consequences. In fact, pulmonary hypertension is associated with an 8- to 10-fold increase in mortality among sarcoidosis patients. Thus, screening, diagnosis and treatment of pulmonary hypertension in sarcoidosis patients is very important.

Unfortunately, there is a lack of guidelines on the management of pulmonary hypertension associated with sarcoidosis. Pulmonary hypertension can be treated with immunosuppressive and pulmonary arterial hypertension (PAH)-targeted therapies, however, their effects on this patient population are understudied.

So, researchers conducted an observational study to determine the effect of these drugs on patients with severe pulmonary hypertension associated with sarcoidosis.

Clinical and hemodynamic data were collected from the French Pulmonary Hypertension Registry, between 2004 and 2015, from 126 patients who were diagnosed with severe sarcoidosis-associated pulmonary hypertension (S-APH). They were characterized by a mean pulmonary artery pressure (mPAP) greater than 35 mmHg, or mPAP 25–35 mmHg, with cardiac index of less than 2.5 L·min⁻¹·m⁻².

Among the 126 patients analyzed in the study, 97 received PAH-targeted therapy. Immunosuppressive therapy was either initiated or increased in 33 patients after a pulmonary hypertension diagnosis.

After four months of PAH-targeted therapy, researchers found that the pulmonary vascular resistance in patients decreased (from 9.7 to 6.9), which is directly related to a decrease in pulmonary hypertension. However, there was no significant improvement in exercise capacity, which is an outcome measure often used in PH.

This indicates that while there are short-term improvements of pulmonary hypertension, the results do not translate to improved exercise capacity.

In the group analyzed, there were 11 patients treated with immunosuppressive therapy only, and among them there was an improvement in blood flow in four patients. This suggests there may be a subset of patients who could benefit from immunosuppressive therapy.

Unfortunately, despite the improvements seen in some patients, after a median follow-up of 28 months, 39 patients needed their PAH-targeted therapy dose to be increased, nine underwent lung transplantation, and 42 patients died.

The survival rate of patients with S-APH at 1 year was 93 percent, 74 percent at 3 years, and 55 percent at 5 years.

“Our large study of severe S-APH confirms that PAH-targeted therapy improves short-term pulmonary hemodynamics without improving exercise capacity. Corticosteroids or immunosuppressive therapy may improve hemodynamics in selected patients. The long-term survival remains poor, which makes lung transplantation a reasonable option for eligible patients” the team concluded.