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Clinical manifestations and diagnosis of cardiac sarcoidosis

Authors
Ron Blankstein, MD
Garrick C. Stewart, MD MPH
Section Editors
Hugh Calkins, MD
William J McKenna, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

The clinical presentation of cardiac sarcoidosis (CS) ranges from an incidentally discovered condition to heart failure and sudden death.

The diagnosis of CS is difficult to establish, and as a result, CS is often under-recognized in clinical practice [1]. CS most often occurs as a manifestation of systemic sarcoidosis, although isolated CS can occur in patients who do not have evidence of sarcoidosis in other organs [2]. Yet, commonly used clinical criteria require the diagnosis of extracardiac disease in order to establish the diagnosis of CS in the absence of having a positive endomyocardial biopsy. While endomyocardial biopsy provides a high specificity for diagnosing CS, this invasive test has a limited sensitivity. Furthermore, there is limited understanding of disease progression and a lack of consensus on the optimal methods for disease detection.

This topic will discuss the clinical manifestations of CS and provide an overview of how to evaluate patients with suspected CS. Management and prognosis of CS and diagnosis and management of systemic sarcoidosis are discussed separately. (See "Management and prognosis of cardiac sarcoidosis" and "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Extrapulmonary manifestations of sarcoidosis" and "Pathology and pathogenesis of sarcoidosis" and "Treatment of pulmonary sarcoidosis: Initial therapy with glucocorticoids" and "Treatment of pulmonary sarcoidosis: Disease refractory to glucocorticoid therapy".)

PREVALENCE

The true prevalence of CS remains unknown and is potentially underestimated since many individuals with CS may have nonspecific symptoms or subclinical disease. CS can affect patients of all racial backgrounds and ages, with an average age at presentation of approximately 50 years old [3].

The prevalence of CS among patients with systemic sarcoidosis has been reported to be 20 to 27 percent in the United States and as high as 58 percent in Japan [4-6]. Yet, autopsy studies indicate that subclinical cardiac involvement may be present in up to 70 percent of cases. In the United States, 13 to 25 percent of deaths from sarcoidosis have been attributed to CS, while in Japan, 47 to 85 percent of deaths from sarcoidosis have been attributed to cardiac involvement [7].

                                                  
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Literature review current through: Nov 2017. | This topic last updated: Dec 11, 2017.
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