Predictors of Treatment Response in Cardiac Sarcoidosis

Quick Takes

  • In a cohort of individuals with proven or suspected cardiac sarcoidosis, who had PET studies before and after steroid initiation, 71% of patients showed complete or partial treatment response at follow-up scan (p = 0.04).
  • Outcomes based on treatment response status—complete treatment response vs. partial response vs. no response—were not significantly different at median follow-up of 4.7 years.

Study Questions:

What is the effect of immunosuppressive therapy and biopsy status to achieve complete treatment response, partial treatment response, or no response on myocardial fluorodeoxyglucose (FDG)–positron emission tomography (PET)/computed tomography (CT) in patients with suspected cardiac sarcoidosis?

Methods:

This study included patients who were steroid treatment naive, had evidence of myocardial FDG at baseline, and underwent repeat PET imaging after prednisone initiation. Follow-up data on death, sustained ventricular arrhythmias, and heart failure admissions were collected.

Results:

There were 83 patients among which 61% had biopsy-confirmed sarcoidosis (11% cardiac biopsy and 50% extracardiac biopsy positive). Overall, 71% of the entire cohort showed complete or partial treatment response at follow-up scan (p = 0.04). Agreement between visual and quantitative assessment of treatment response was excellent. In patients receiving prednisone only, the highest rates of treatment response were observed in patients initiated on moderate or high dose (p < 0.01). After a median follow-up of 4.7 years, patients who were biopsy-proven and those with preserved left ventricular function were less likely to experience major adverse cardiac events. Outcomes based on treatment response status were not significantly different.

Conclusions:

The authors concluded that among patients with suspected sarcoidosis, a favorable response to prednisone was more common when using moderate- to high-intensity dose. Biopsy-proven individuals and those with preserved systolic function were less likely to experience adverse outcomes during follow-up.

Perspective:

Despite widespread use of steroids as first-line treatment for cardiac sarcoidosis, the optimal intensity and duration of the immunosuppression has not been determined, and the optimal method and frequency of monitoring of the disease activity remains unclear. The authors of the present manuscript showed that moderate- to high-dose prednisone results in a higher chance of complete or partial treatment response than lower-dose prednisone, which would be expected.

Individuals without biopsy confirmation were more likely to experience adverse outcomes, but PET-ascertained treatment response did not translate into significant differences in outcomes at follow-up. Nonbiopsy-proven patients had similar treatment response as those with a positive biopsy. The current study suggests that an empiric start of prednisone at a dose between 30 and 40 mg/day appears reasonable, and it probably should be followed by a slower rather than rapid taper. Future randomized studies are needed to further clarify the optimal intensity and duration of steroid taper.

Clinical Topics: Arrhythmias and Clinical EP, Cardio-Oncology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Interventions and Imaging, Computed Tomography, Nuclear Imaging

Keywords: Arrhythmias, Cardiac, Biopsy, Cardiotoxicity, Diagnostic Imaging, Fluorodeoxyglucose F18, Heart Failure, Immunosuppression, Positron Emission Tomography Computed Tomography, Prednisone, Sarcoidosis, Tomography, X-Ray Computed, Treatment Outcome, Ventricular Function, Left


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