Effect of Immunosuppressive Therapy and Biopsy Status in Monitoring Therapy Response in Suspected Cardiac Sarcoidosis

JACC Cardiovasc Imaging. 2022 Nov;15(11):1944-1955. doi: 10.1016/j.jcmg.2022.05.015. Epub 2022 Aug 17.

Abstract

Background: Patients with suspected cardiac sarcoidosis frequently undergo fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) imaging to assess disease activity at baseline and after treatment initiation.

Objectives: This study investigated the effect of immunosuppressive therapy and biopsy status to achieve complete treatment response (CTR), partial treatment response (PTR), or no response (NR) on myocardial FDG-PET/CT.

Methods: This study analyzed 83 patients with suspected cardiac sarcoidosis (aged 53 ± 1.8 years, 71% were male, 69% were White, 61% had a history of biopsy-confirmed sarcoidosis) who were treatment naive, had evidence of myocardial FDG at baseline, and underwent repeat PET imaging after treatment initiation. CTR was graded visually, and PTR/NR were measured both visually and quantitatively using the total glycolytic activity. Patients were also evaluated for the occurrence of death, sustained ventricular arrhythmias, and heart failure admissions.

Results: Overall, 59 patients (71%) achieved CTR/PTR (30%/41%) at follow-up scan (P = 0.04). Total glycolytic activity and visual estimate of PTR/NR had excellent agreement (κ = 0.86 [95% CI: 0.72-0.99]; P < 0.0001). In patients receiving prednisone only, the highest rates of CTR/PTR were observed in patients initiated on moderate or high dose (P < 0.01). In a regression model, moderate prednisone start dose (P = 0.03) was more strongly associated with achieving CTR/PTR than was high prednisone start dose. However, the latter patients were tapered faster between start dose and follow-up scan (P < 0.01). After a median follow-up of 4.7 (IQR: 3.1-7.8) years, patients who were biopsy-proven (vs non-biopsy-proven; P = 0.029) and with preserved left ventricular function (P = 002) were less likely to experience major adverse cardiac events. Outcomes based on treatment response status (CTR vs PTR vs NR; P = 0.23) were not significantly different.

Conclusions: Among patients with suspected sarcoidosis and evidence of myocardial inflammation, treatment response by serial FDG-PET was variable, but a favorable response was more common when using moderate-to-high intensity prednisone dose. Biopsy-proven individuals and those with preserved systolic function were less likely to experience adverse outcomes during follow-up.

Keywords: positron emission tomography; sarcoidosis; treatment response.

MeSH terms

  • Cardiomyopathies* / diagnostic imaging
  • Cardiomyopathies* / drug therapy
  • Cardiomyopathies* / pathology
  • Female
  • Fluorodeoxyglucose F18
  • Humans
  • Immunosuppression Therapy
  • Male
  • Myocarditis*
  • Positron Emission Tomography Computed Tomography
  • Positron-Emission Tomography / methods
  • Predictive Value of Tests
  • Prednisone
  • Radiopharmaceuticals
  • Sarcoidosis* / diagnostic imaging
  • Sarcoidosis* / drug therapy
  • Sarcoidosis* / pathology

Substances

  • Fluorodeoxyglucose F18
  • Radiopharmaceuticals
  • Prednisone