Challenges in Cardiac and Pulmonary Sarcoidosis

Highlights

• Cardiac and pulmonary involvement in sarcoidosis is associated with greater morbidity and mortality than other manifestations of the disease.

• Diagnosis of sarcoidosis should consider the clinical presentation, histological evidence of granulomatous inflammation, and exclusion of alternative granulomatous diseases.

• The lack of validated diagnostic tests or biomarkers of disease activity has hindered research into disease mechanisms, therapeutic opportunities, or assessment of the response to treatment, and should be a focus of future investigations.

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.