What type of different clinical manifestations can cardiac sarcoidosis present?

Abstract Funding Acknowledgements Type of funding sources: None. Background Overt cardiac involvement is reported in 5% of patients with sarcoidosis, although autopsy and imaging studies suggest higher prevalence, worldwide variation. The role of 18F-fluorodeoxyglucose positron emission tomography ([18F]FDG-PET) in non-invasive diagnosis and follow-up has increased in the last decade. Purpose Our goal is to describe the prevalence, clinical manifestations and outcomes of cardiac sarcoidosis (CS), diagnosed through [18F]FDG-PET, in a southern European population. Methods We included all patients with histological diagnosis of extracardiac sarcoidosis screened with [18F]FDG-PET between 2009 and 2020. We collected data on clinical manifestations, cardiac magnetic resonance (CMR) results, and mortality outcomes and compared those with and without cardiac involvement. We applied the criteria for the diagnosis of CS from Heart Rhythm Society. Results Of the 400 patients screened with [18F]FDG-PET, 128 had a histological diagnosis of extracardiac sarcoidosis (54.7% females, mean age 51.0 ± 14.2 years). None underwent endomyocardial biopsy. Ten patients had a pattern of [18F]FDG uptake consistent with CS defined as diffuse (n = 5), focal (n = 3), and focal on diffuse (n = 2). Of the 128 patients, 14 also underwent CMR, which identified 2 subjects with positive findings in both modalities and 3 additional patients: focal (n = 1), multifocal mid-wall (n = 2), focal mid-wall (n = 2), and multifocal subepicardial (n = 1) delayed gadolinium enhancement. Overall, 13 patients (10.2%) fulfilled the criteria for probable CS (53.8% female, mean age 56.2 ± 12.6 years), all with multiorgan involvement, mostly lung and lymph nodes (each 92%), followed by skin and central nervous system (each 15%). Median left ventricle ejection fraction was 62% [55-65] and there were cardiac manifestations of CS in 6 patients (46%): sick sinus syndrome (n = 2), complete heart block (n = 1), frequent premature ventricular complexes (n = 1), ventricular tachycardia plus heart failure (n = 1), and bifascicular block plus heart failure (n = 1). Eleven patients (85%) with probable CS were medicated with immunosuppressant drugs: corticosteroids (n = 9), methotrexate (n = 4), and azathioprine (n = 2). Four patients with previous [18F]FDG screening were revaluated after treatment, each showing no cardiac uptake. After a mean follow-up of 4.0 ± 1.0 years, mortality was three-fold higher in patients with cardiac involvement, despite the absence of statistical significance (15% vs. 5%, P = 0.151). Conclusions In a southern European population with histological extracardiac sarcoidosis, the prevalence of cardiac involvement was 10.2%, most asymptomatic. [18F]FDG-PET improves the diagnostic yield and plays an important role in monitoring response to therapy. The higher mortality trend in those with CS needs to be ascertained in longer follow-up.

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Sarcoidosis

10.2310/im.1209 ◽

2020 ◽

Author(s):

Robert P Baughman ◽

Mary Beth Scholand

Keyword(s):

Cardiac Sarcoidosis ◽

Systemic Disease ◽

Clinical Manifestations ◽

Host Factors ◽

Resonance Imaging ◽

Noncaseating Granuloma ◽

Symptomatic Disease ◽

Contrast Enhanced ◽

Löfgren Syndrome ◽

Noncaseating Granulomas

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 7 tables, and 127 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

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Sarcoidosis

10.2310/fm.1209 ◽

2020 ◽

Author(s):

Robert P Baughman ◽

Mary Beth Scholand

Keyword(s):

Cardiac Sarcoidosis ◽

Systemic Disease ◽

Clinical Manifestations ◽

Host Factors ◽

Resonance Imaging ◽

Noncaseating Granuloma ◽

Symptomatic Disease ◽

Contrast Enhanced ◽

Löfgren Syndrome ◽

Noncaseating Granulomas

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 3 tables, and 126 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

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Cardiac sarcoidosis: A review on the work-up and management

Open Medicine ◽

10.2478/s11536-012-0095-8 ◽

2013 ◽

Vol 8 (1) ◽

pp. 107-116

Author(s):

Boris Solun ◽

Dana Marcoviciu ◽

Yulia Belnik ◽

Tamar Azran ◽

Dror Dicker ◽

...

Keyword(s):

Sudden Death ◽

Cardiac Sarcoidosis ◽

Ventricular Tachyarrhythmia ◽

Clinical Manifestations ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Diagnostic Tools ◽

Unknown Etiology ◽

Ventricular Ejection Fraction ◽

Electrocardiographic Monitoring

AbstractSarcoidosis is a multisystem granulomatous disease of unknown etiology and with variable presentation. Skin, lymph nodes, lungs, eyes and the central nervous system are mostly involved. Cardiac sarcoidosis (CS) is a rare condition with clinical manifestations in about 5% of patients. Since it increases the risk of acute cardiac failure, ventricular arrhythmia, conduction disturbances and even sudden death, it aggravates markedly the prognosis. The early diagnosis of CS is difficult, requiring the use of diagnostic tools such as electrocardiographic monitoring, two-dimensional echocardiography, radionuclide scan, cardiac magnetic resonance imaging, positron emission tomography and endomyocardial biopsy. Once the diagnosis of CS is established, there is a need for early corticosteroids treatment, with or without immunosuppressive therapy, to prevent deterioration of cardiac function. In patients with refractory ventricular tachyarrhythmia, markedly reduced left ventricular ejection fraction and high risk of sudden death, prophylactic insertion of a pacemaker or implantable defibrillator is recommended. We had the opportunity to treat a patient with CS and to review the currently accepted diagnostic and treatment approach.

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In Which Patients with Sarcoidosis Is FDG PET/CT Indicated?

Journal of Clinical Medicine ◽

10.3390/jcm9030890 ◽

2020 ◽

Vol 9 (3) ◽

pp. 890 ◽

Cited By ~ 5

Author(s):

Ruth G.M. Keijsers ◽

Jan C. Grutters

Keyword(s):

Fdg Pet ◽

Cardiac Sarcoidosis ◽

Clinical Manifestations ◽

Emission Tomography ◽

Diagnostic Process ◽

Inflammatory Lesions ◽

Positron Emission ◽

Pet Ct ◽

Conventional Tests ◽

Fdg Pet Ct

Sarcoidosis is a granulomatous disease of which the etiology remains unknown. The diverse clinical manifestations may challenge clinicians, particularly when conventional markers are inconclusive. From various studies, it has become clear that fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT aids in sarcoidosis care. In this article, an update on FDG PET/CT in sarcoidosis is provided. The use of FDG PET/CT in the diagnostic process of sarcoidosis is explained, especially in determining treatable inflammatory lesions in symptomatic patients with indecisive conventional tests. Furthermore, FDG PET/CT for evaluating the potential benefit of additional inflammatory treatment is described and its use in cardiac sarcoidosis is highlighted.

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Management of Cardiac Sarcoidosis in 2020

Arrhythmia & Electrophysiology Review ◽

10.15420/aer.2020.09 ◽

2020 ◽

Vol 9 (4) ◽

pp. 182-188

Author(s):

Nisha Gilotra ◽

David Okada ◽

Apurva Sharma ◽

Jonathan Chrispin

Keyword(s):

Heart Failure ◽

Heart Block ◽

Ventricular Arrhythmias ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Management Strategies ◽

Clinical Manifestations ◽

Future Directions ◽

Inflammatory Phase

Sarcoidosis is an inflammatory granulomatous disease that can affect any organ. Up to one-quarter of patients with systemic sarcoidosis may have evidence of cardiac involvement. The clinical manifestations of cardiac sarcoidosis (CS) include heart block, atrial arrhythmias, ventricular arrhythmias and heart failure. The diagnosis of CS can be challenging given the patchy infiltration of the myocardium but, with the increased availability of advanced cardiac imaging, more cases of CS are being identified. Immunosuppression with corticosteroids remains the standard therapy for the acute inflammatory phase of CS, but there is an evolving role of steroid-sparing agents. In this article, the authors provide an update on the diagnosis of CS, including the role of imaging; review the clinical manifestations of CS, namely heart block, atrial and ventricular arrhythmias and heart failure; discuss updated management strategies, including immunosuppression, electrophysiological and heart failure therapies; and identify the current gaps in knowledge and future directions for cardiac sarcoidosis.

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Cardiac sarcoidosis manifestations

Clinical review for general practice ◽

10.47407/kr2020.1.1.00007 ◽

2020 ◽

Vol 1 (1) ◽

pp. 42-48

Author(s):

Olga Karpova ◽

◽

Leonid Dvoretsky ◽

Keyword(s):

Case Report ◽

Early Diagnosis ◽

Cardiac Involvement ◽

Cardiac Sarcoidosis ◽

Clinical Manifestations ◽

Pulmonary Sarcoidosis ◽

Cardiac Lesion ◽

Cardiac Symptoms ◽

Heart Lesion ◽

Possible Cause

Patient with pulmonary sarcoidosis and signs of cardiac involvement under the lung symptoms improvement: case report. The possible cause of cardiac symptoms (granulomatous inflammatory response, myocardial fibrosis) is discussed. The data on morbidity, clinical manifestations and cardiac lesion diagnosis in patients with sarcoidosis is reported. The need for the heart lesion signs thorough search for the purpose of early diagnosis and adequate therapy is emphasized.

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