PAH Drugs of Limited Benefit in Sarcoidosis-linked Pulmonary Hypertension, Study Finds

PAH Drugs of Limited Benefit in Sarcoidosis-linked Pulmonary Hypertension, Study Finds

Treatment with medications for pulmonary arterial hypertension (PAH) do not significantly help people with sarcoidosis-associated pulmonary hypertension (PH), researchers from France’s University of Paris-Sud reported.

Their study, Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension,” — published in the European Respiratory Journal — showed that while PAH-drugs improved some disease parameters in the short term, they had no impact on exercise capacity or improved survival.

With this study, likely the largest report of outcomes in patients with sarcoidosis-associated PH, it becomes clear that the prognosis for these patients remains poor.

The research team turned to the French Pulmonary Hypertension Registry and identified 126 patients with sarcoidosis who developed PH.

For half of the patients, the PH diagnosis had come 17 or more years after being diagnosed with sarcoidosis. Most of them, 72 percent, had end-stage sarcoidosis as judged by chest X-rays. In addition, 24 patients had a forced vital capacity (FVC) — the amount of air a person can exhale — less than 50 percent of normal.

PAH medications were used by 77 percent of the group and included both single-drug and combination treatments. The most common were  endothelin receptor blockers such as Tracleer (bosentan) or Letairis (ambrisentan), or phosphodiesterase type 5 inhibitors such as Revatio (sildenafil) or Adcirca (tadalafil).

A small group of 11 patients received only immunosuppressive treatment or corticosteroids, and another 22 were treated with both PAH drugs and immunosuppressives.

Follow-up among those treated with PAH drugs suggested that the medications improved lung blood flow parameters and the severity of heart failure. However, the treatment did not allow patients to improve on a 6-minute walk distance test of exercise capacity.

Immunosuppression mainly improved lung blood flow parameters in patients whose lung blood vessels were compressed by enlarged lymph nodes. But such treatment had no impact on PH functional classification or exercise capacity.

Patients treated with PAH drugs also lived for about as long as those who were not treated with these therapies.

When examining factors that predicted a poor survival, researchers found that poor exercise capacity was the one most strongly linked to a poor prognosis. The data also indicated that a lung transplant is a reasonable option for patients with sarcoidosis-associated lung hypertension.

“In conclusion, our large study of severe S-APH [sarcoidosis-associated PH] confirms that PAH-targeted therapy improves short-term pulmonary hemodynamics without improving exercise capacity,” the team concluded. “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.”

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