Nonsteroidal immunosuppressants may be cardiac sarcoidosis option
Treatment reduced corticosteroid need, lesion activity for some patients
Nonsteroidal immunosuppressants may be a treatment option for people with cardiac sarcoidosis who don’t respond well to corticosteroids or have side effects from using them, a Japanese study suggests.
While nonsteroidal immunosuppressants reduced the need for corticosteroids and how active sarcoidosis lesions were for some patients, more research is needed to determine how safe they are and how well they work.
The study, “Cardiac sarcoidosis treated with nonsteroidal immunosuppressive therapy,” was published in IJC Heart & Vasculature. It was partially funded by Novartis, which markets the nonsteroidal immunosuppressant cyclosporine under the names Sandimmune and Neoral.
Cardiac sarcoidosis is a type of sarcoidosis that occurs when granulomas, or small clusters of inflammatory cells, affect the heart and interfere with how blood is pumped out to the body, leading to irregular heartbeats and heart failure.
First-line treatment usually includes corticosteroids, which reduce inflammation, but don’t always work well for every patient. They can also cause serious side effects, such as weight gain and diabetes when taken at high doses or after prolonged use.
Another option for treating cardiac sarcoidosis?
To see if nonsteroidal immunosuppressants might be an option for cardiac sarcoidosis, the researchers drew on data from the ILLUMINATE-CS study (UMIN000034974), a registry of people with cardiac sarcoidosis in Japan. Of 512 patients, 426 were receiving corticosteroids and 26 nonsteroidal immunosuppressants. The rest didn’t receive either treatment, or else their data were missing, and were excluded from the study.
Patients took nonsteroidal immunosuppressants for various reasons, such as ongoing inflammation of the heart, side effects from corticosteroids, irregular heartbeats from the heart’s lower chambers, and atrioventricular block, which causes the heart to beat more slowly than it should. Two patients had worsening sarcoidosis outside the heart.
Of the 26 patients on nonsteroidal immunosuppressants, 13 (50%) were men. The mean age at diagnosis was 60.3 and the most common manifestations of cardiac sarcoidosis at that time were impaired wall motions (42.3%), which refers to changes in how well the heart muscle contracts, and atrioventricular block (34.6%), a condition where the electrical signals that normally travel from the top part of the heart to the bottom get disrupted or blocked.
The most commonly used nonsteroidal immunosuppressant was methotrexate, by 20 patients. Three patients were on azathioprine, two on cyclosporine and two on cyclophosphamide. Besides reducing inflammation, methotrexate, azathioprine, and cyclophosphamide also stop cells from growing.
When nonsteroidal immunosuppressants were added to their treatment regimens, 14 patients were able to reduce their corticosteroid doses, which may help reduce side effects. None stopped taking corticosteroids, however.
Among the 14 patients treated with nonsteroidal immunosuppressants for persistent heart inflammation, half (50%) showed reduced fluorodeoxyglucose uptake in a PET scan. This radioactive sugar molecule is typically absorbed more by rapidly growing granulomas than healthy cells for energy production. Therefore, a decrease in fluorodeoxyglucose uptake suggests diminished inflammation after treatment.
After about five years of follow-up, 11 patients (42.3%) had negative outcomes, including death from any cause in five (19.2%), worsening heart failure with hospitalization in five (19.2%), and fatal irregular heartbeats in four (15.4%), suggesting nonsteroidal immunosuppressants didn’t fully control the disease in some cases.
“Nonsteroidal immunosuppressive therapy may be a possible treatment option for patients who are not stabilized with corticosteroids alone or develop corticosteroid side effects,” wrote the researchers, who noted more research was needed to determine who may best benefit from them.
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