Key result
Cardiac sarcoidosis is clinically manifest in ~5% of patients, though occult involvement affects up to ~25%.
Left ventricular dysfunction is the primary prognostic predictor in clinically manifest cardiac sarcoidosis, which is typically managed with immunosuppression and implantable cardioverter-defibrillators.
Clinically manifest cardiac involvement occurs in perhaps 5% of patients with sarcoidosis. The 3 principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. An estimated 20% to 25% of patients with pulmonary/systemic sarcoidosis have asymptomatic cardiac involvement (clinically silent disease). In 2014, the first international guideline for the diagnosis and management of CS was published. In patients with clinically manifest CS, the extent of left ventricular dysfunction seems to be the most important predictor of prognosis. There is controversy in published reports as to the outcome of patients with clinically silent CS. Despite a paucity of data, immunosuppression therapy (primarily with corticosteroids) has been advocated for the treatment of clinically manifest CS. Device therapy, primarily with implantable cardioverter-defibrillators, is often recommended for patients with clinically manifest disease.
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Birnie et al. (2016) conducted a review in Cardiac Sarcoidosis. Clinically manifest cardiac sarcoidosis occurs in approximately 5% of patients with sarcoidosis, while an estimated 20% to 25% have asymptomatic cardiac involvement.
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