Challenges in Cardiac and Pulmonary Sarcoidosis: JACC State-of-the-Art Review

J Am Coll Cardiol. 2020 Oct 20;76(16):1878-1901. doi: 10.1016/j.jacc.2020.08.042.

Abstract

Sarcoidosis is a complex disease with heterogeneous clinical presentations that can affect virtually any organ. Although the lung is typically the most common organ involved, combined pulmonary and cardiac sarcoidosis (CS) account for most of the morbidity and mortality associated with this disease. Pulmonary sarcoidosis can be asymptomatic or result in impairment in quality of life and end-stage, severe, and/or life-threatening disease. The latter outcome is seen almost exclusively in those with fibrotic pulmonary sarcoidosis, which accounts for 10% to 20% of pulmonary sarcoidosis patients. CS is problematic to diagnose and may cause significant morbidity and death from heart failure or ventricular arrhythmias. The diagnosis of CS usually requires surrogate cardiac imaging biomarkers, as endomyocardial biopsy has relatively low yield, even with directed electrophysiological mapping. Treatment of CS is often multifactorial, involving a combination of antigranulomatous therapy and pharmacotherapy for cardiac arrhythmias and/or heart failure in addition to device placement and cardiac transplantation.

Keywords: biomarkers; cardiac sarcoidosis; imaging; pulmonary sarcoidosis.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Cardiomyopathies / diagnostic imaging*
  • Cardiomyopathies / drug therapy
  • Cardiomyopathies / metabolism
  • Humans
  • Magnetic Resonance Imaging, Cine / methods
  • Review Literature as Topic
  • Sarcoidosis / diagnostic imaging
  • Sarcoidosis / metabolism
  • Sarcoidosis, Pulmonary / diagnostic imaging*
  • Sarcoidosis, Pulmonary / drug therapy
  • Sarcoidosis, Pulmonary / metabolism

Substances

  • Antibodies, Monoclonal