Key result
Fibrotic pulmonary sarcoidosis affects ~15% of patients and is linked to severe, life-threatening disease.
Cardiac and pulmonary sarcoidosis present significant diagnostic and therapeutic challenges, often requiring a multifactorial approach including imaging biomarkers and combined pharmacotherapy.
Sarcoidosis is a complex disease with heterogeneous clinical presentations that can affect virtually any organ. While 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, end-stage severe and/or life-threatening disease. The latter outcome is seen almost exclusively in those with fibrotic pulmonary sarcoidosis, which accounts for 10–20 percent of pulmonary sarcoidosis patients. CS is problematic to diagnose and may cause significant morbidity and death from heart failure (HF) or ventricular arrhythmias. The diagnosis of CS usually requires surrogate cardiac imaging biomarkers as endomyocardial biopsy (EMBx) has relatively low yield, even with directed electrophysiologic mapping. Treatment of CS is often multifactorial involving a combination of anti-granulomatous therapy and pharmacotherapy for cardiac arrhythmias and/or HF in addition to device placement and cardiac transplantation.
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Trivieri et al. (2020) conducted a review in Cardiac and Pulmonary Sarcoidosis. Fibrotic pulmonary sarcoidosis accounts for 10-20% of pulmonary sarcoidosis patients and is associated with end-stage severe and life-threatening disease.
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