Cardiac Sarcoidosis: What to Do When Second-Line Steroid-Sparing Therapy Fails to Clear Myocardial Inflammation on FDG-PET
This protocol addresses the specific situation in which a combination of corticosteroids and a steroid-sparing agent has not produced the required reduction in active myocardial inflammation—confirmed on reassessment cardiac FDG-PET—and escalation to a third-line approach is needed.
The prior regimen paired corticosteroids with a steroid-sparing agent: methotrexate, mycophenolate, azathioprine, or leflunomide. The treatment target was minimal residual or resolved myocardial inflammation on cardiac FDG-PET at 2 to 6 months. This protocol is indicated when that target was not achieved.
This protocol introduces tumor necrosis factor-α–targeted therapy as an additional third-line agent. Specific agent selection, timing, and which patient populations require particular caution are detailed in the complete regimen—available via the link below.
Reduction in the degree of active granulomatous inflammation in the myocardium, assessed on cardiac FDG-PET.
References
DOI: 10.1161/CIR.0000000000001240- If there is evidence of ongoing inflammation on follow-up FDG-PET, then tumor necrosis factor-α–targeted therapy with infliximab or adalimumab can be considered as a third-line agent.
- Tumor necrosis factor-α–targeted therapy should be used cautiously in individuals with HF with reduced ejection fraction and New York Heart Association class III to IV symptoms because prior trials investigating these agents in HF suggested potential harm in patients with HF.
- The response to treatment is measured in 2 ways: (1) improvement or resolution of the clinical presentation of arrhythmias, heart block, or HF and (2) reduction in the degree of active granulomatous inflammation in the myocardium.