Author + information
- Received February 26, 2020
- Revision received August 12, 2020
- Accepted August 13, 2020
- Published online October 12, 2020.
- Maria Giovanna Trivieri, MD, PhDa,b,∗ (, )@mgtrivieri,
- Paolo Spagnolo, MD, PhDc,
- David Birnie, MDd,
- Peter Liu, MDd,
- Wonder Drake, MDe,
- Jason C. Kovacic, MD, PhDb,
- Robert Baughman, MDf,
- Zahi A. Fayad, PhDa@zahifayad and
- Marc A. Judson, MDg
- aThe BioMedical Engineering and Imaging Institute (BMEII), Icahn School of Medicine at Mount Sinai, New York, New York
- bCardiovascular Institute, Icahn School of Medicine at Mount Sinai, New York, New York
- cSection of Respiratory Diseases, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padua, Italy
- dDivision of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada
- eDivision of Infectious Disease, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee
- fDepartment of Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio
- gDivision of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, New York
- ↵∗Address for correspondence:
Dr. Maria Giovanna Trivieri, Icahn School of Medicine at Mount Sinai, 1 Gustave Levy, P.O. Box 1030, New York, New York 10029.
• 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.
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.
This work was supported by National Institutes of Health grants NIH/NHLBI R01HL071021 (to Dr. Fayad), R01 HL 117074 (to Dr. Drake), and KL2 TR001435 (to Dr. Trivieri). The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC author instructions page.
- Received February 26, 2020.
- Revision received August 12, 2020.
- Accepted August 13, 2020.
- 2020 American College of Cardiology Foundation
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