Sarcoidosis is an inflammatory disease of unknown etiology that affects multiple systems. Cardiac sarcoidosis is diagnosed approximately 2-5% with other systemic sarcoid; however, we now know by way of autopsy that cardiac sarcoidosis may be as high as 20-30% in the United States.1 The prevalence of sarcoidosis is 3.8 times greater in African Americans compared with Caucasians in the United States and Europe.2 The prevalence of cardiac sarcoidosis maybe as high as 58% in Japanese patients and the cause of death in 85% of Japanese patients with sarcoid.3,4 The prognosis for cardiac sarcoidosis is poor, and patients can deteriorate quickly. Signs and symptoms of cardiac sarcoidosis may include chest pain, palpitations, syncope, presyncope, fatigue, shortness of breath, and edema. Cardiac sarcoid is most commonly manifested by atrioventricular disease, atrial arrhythmias, ventricular arrhythmias, and heart failure. When cardiac sarcoidosis is suspected, the evaluation includes a detailed history and physical exam, electrocardiogram and Holter monitor to detect arrhythmias, and echocardiogram determine wall size and function. Abnormalities in initial testing should be followed up with F-fluorodeoxyglucose positron emission tomography scan and cardiac magnetic resonance to assist with determining if cardiac sarcoidosis is the cause for the abnormality. The gold standard for the diagnosis of cardiac sarcoidosis is endometrial biopsy, but it can be difficult to get affected tissue.
The primary treatment of sarcoidosis is corticosteroids, but immunosuppressive agents should be considered for select patients. Other treatment strategies include the following second-line treatments:
- Mycophenolate mofetil
The tumor necrosis factor alpha antagonists infliximab and adalimumab (often reserved for refractory disease) can also be considered as third-line treatment.5 Non-immunosuppressive medications targeting specific cardiac targets of sarcoidosis should also be used, including guideline-directed medical therapies for heart failure. Antiarrhythmic medications maybe warranted if the patient presents with atrial or ventricular arrhythmias. The most frequent cause of death in cardiac sarcoidosis is due to ventricular arrhythmias, and appropriate device therapy and/or procedure should be considered as indicated. In 2014, the Heart Rhythm Society developed an expert consensus statement on arrhythmia management of cardiac sarcoidosis. The document includes the following guidance and recommendations for cardiac sarcoidosis: treat extracardiac sarcoidosis and screen for cardiac involvement, manage arrhythmias for cardiologist and electrophysiologist, stratify sudden death risk, consider appropriate implantable cardioverter-defibrillator indications, establish criteria for diagnosis, and identify those gaps in data to help guide future research.6 Cardiac transplant may be the only option for patients with advanced cardiac sarcoidosis.
Many programs across the country are developing sarcoid or inflammatory disease clinics. Given that sarcoidosis is a multisystem disease, it requires a multidiscipline approach. Some disciplines often involve a pulmonologist, heart failure specialist, electrophysiologist, neurologist, radiologist, ophthalmologist, dermatologist, and rheumatologist. The disciplines involved may vary based on the specific needs of the patient. With the involvement of multiple disciplines, the importance of team-based care becomes extremely important. In fact, the World Association of Sarcoidosis and other Granulomatous Disorders Sarcoidosis Center of Excellence has been established and recognizes 25 institutions for the designation of "multidisciplinary teams of specialized medical and paramedical professionals in a shared specialized facility for years and that provides leadership, best practices, research, support and/or education for sarcoidosis patients and professionals."7 Advance practice providers are making significant contributions to these teams because they provide a unique skill set and expertise specifically related to assessment skills, care coordination, expertise in evidence-base care, chronic care management, interpersonal collaboration, and team-based care.
- What is Sarcoidosis? (Foundation for Sarcoidosis Research website). 2021. Available at https://www.stopsarcoidosis.org/about/. Accessed March 18, 2021.
- Westney GE, Judson MA. Racial and ethnic disparities in sarcoidosis: from genetics to socioeconomics. Clin Chest Med. 2006;27:453-62.
- Hulten E, Aslam S, Osborne M, Abbasi S, Bittencourt MS, Blankstein R. Cardiac sarcoidosis-state of the art review. Cardiovasc Diagn Ther 2016;6:50-63.
- Sekhri V, Sanal S, Delorenzo LJ, Aronow WS, Maguire GP. Cardiac sarcoidosis: a comprehensive review. Arch Med Sci 2011;7:546-54.
- Gilotra N, Okada D, Sharma A, Chrispin J. Management of Cardiac Sarcoidosis in 2020. Arrhythm Electrophysiol Rev 2020;9:182-8.
- Birnie DH, Sauer WH, Bogun F, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm 2014;11:1305-23.
- WASOG Sarcoidosis Centers of Excellence (World Association for Sarcoïdosis and Other Granulomatous Disorders website). 2021. Available at https://www.wasog.org/about/wasog-centers-of-excellence.html. Accessed March 17, 2021.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Dyslipidemia, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and Heart Failure, Lipid Metabolism, Novel Agents, Statins, Acute Heart Failure, Heart Transplant, Interventions and Imaging, Computed Tomography, Nuclear Imaging
Keywords: Patient Care Team, Sarcoidosis, Azathioprine, Mycophenolic Acid, Methotrexate, Hydroxychloroquine, Tumor Necrosis Factor-alpha, Defibrillators, Implantable, Immunosuppressive Agents, African Americans, Leadership, Cause of Death, Arrhythmias, Cardiac, Electrocardiography, Heart Failure, Heart Transplantation, Positron-Emission Tomography, Syncope, Adrenal Cortex Hormones, Chest Pain, Physical Examination, Magnetic Resonance Spectroscopy, Fatigue, Death, Sudden, Death, Sudden, Cardiac, Dyspnea, Reference Standards, Edema
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