Context: Sarcoidosis is a granulomatous disease of unclear etiology. It is not commonly fatal, but when sarcoidosis is fatal, it is most often from cardiac involvement and when sarcoidosis involves the heart, it frequently causes death. The disease presents diagnostic challenges both clinically and histologically.
Objectives: To review the histology of cardiac sarcoidosis and the histologic differential diagnosis of cardiac granulomatous disease and to review the epidemiology and gross pathology of cardiac sarcoid as well as discuss current controversies, clinical diagnostic criteria, and proposed mechanisms of pathogenesis.
Data sources: We reviewed the literature searchable on PubMed as well as selected older studies revealed by our review of the recent literature. Photographs were taken from cases on file at the University of Pittsburgh Medical Center (Pittsburgh, Pennsylvania) and Columbia University Medical Center (New York, New York).
Conclusions: Sarcoidosis is a focal or disseminated granulomatous disease that likely represents the final common pathway of various pathogenic insults in a genetically susceptible host. The type of insult may influence the specific sarcoid phenotype. Controversy still abounds, but many areas of investigation around sarcoidosis are yielding exciting discoveries and bringing us closer to a richer understanding of this puzzling disease.