While there no therapies approved for sarcoidosis-associated pulmonary hypertension (SAPH), pulmonary hypertension-specific therapies may help ease right heart dysfunction in a select group of patients, according to results of a large cohort study.
The study, “Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension,” was published in the journal Scientific Reports.
Pulmonary hypertension (PH) is a serious complication of sarcoidosis. It is estimated to affect between 5% and 28% of patients, but this number increases to 74% in patients with advanced sarcoidosis.
PH is associated with heart complications, causing heart enlargement due to the extra effort the cardiac muscle has to make to send blood to the lungs; it can also lead to the formation of blood clots, arrhythmia (irregular heartbeat), and even bleeding.
People with sarcoidosis-associated pulmonary hypertension, or SAPH, are at a higher risk of death, as shown by increased levels of a marker of right heart dysfunction called NT-proBNP.
A team led by researchers at Duke University have now investigated the clinical outcomes of a group of SAPH patients, including how different PH therapies affected their disease progression.
In total, researchers analyzed 95 patients — 76% of them were women, and 86% were African American — with biopsy-confirmed sarcoidosis and an SAPH diagnosis, assessed by right heart catheterization, a procedure where a catheter is passed into a vein in the neck or groin to measure the pressure in the heart and lungs.
Participants’ mean age at time of SAPH diagnosis was 52 years. Of the patients analyzed, 70% had advanced sarcoidosis (stage 4). The patients were followed for up to 11 years.
Most of the SAPH patients were being treated for sarcoidosis with steroids (61%) or non-steroidal immunosuppressive therapy (18%). Additionally, 78% of patients received PH-specific treatment, while the remaining 22% were treatment-free.
Analysis showed that 59% of the patients had moderate or severe heart right ventricular enlargement, and 55% had moderate or severe right ventricular dysfunction. Moreover, initial levels of the peptide NT-proBNP — released when the heart is at effort — were elevated, with a median value of 910 picograms per milliliter.
The median time to hospitalization or death was six months.
The NT-proBNP levels were higher in SAPH patients who died or were hospitalized, but decreased after PH therapy — a mean reduction of 51.2%. In contrast, PH therapy had no effects on the six-minute walk distance test, an exercise capacity test, which measures the maximum distance an individual is able to walk over six minutes on a hard, flat surface.
According to the team, “only follow-up NT-proBNP was associated with all-cause hospitalization or mortality.”
Patients who began PH therapy, either intravenous, inhaled, or oral combination treatment, were much more likely to experience a clinical event in the follow-up period.
However, since these PH-specific therapies were well-tolerated and actually led to lower NT-proBNP levels, “these findings are less likely related to adverse drug effect, and instead reflects a sicker subgroup of SAPH patients,” the researchers wrote.
These findings suggest that the “use of PH-specific therapy may be helpful in selected patients with SAPH,” they concluded, adding that “prospective trials are needed to characterize responses to PH-specific therapy in this subset of patients with SAPH.”