Affiliation:
1. Department of Cardiology, Campus Benjamin Franklin, Charité—Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin , Hindenburgdamm 30, 12203 Berlin , Germany
2. Department of Gastroenterology, Infectious Diseases and Rheumatology, Campus Benjamin Franklin, Charité—Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin , Hindenburgdamm 30, 12203 Berlin , Germany
Abstract
Abstract
Background
Sarcoidosis is a granulomatous multi-organ disease of unknown aetiology. Despite being relatively rare, cardiac sarcoidosis constitutes a very important manifestation of sarcoidosis, as its symptoms regularly precede or occur in isolation of more prevalent ones, and as it is the main driver of mortality in systemic sarcoidosis.
Case summary
We present the case of a 37-year-old woman, in which clinically isolated cardiac sarcoidosis revealed widespread systemic sarcoidosis. Apart from constitutional symptoms and strong recurrent dizziness (i.e. near-syncopes), which persisted for multiple years already, our patient initially presented with complex conduction abnormalities, including a right bundle branch block, left anterior hemi-block, and atrioventricular block °1. Following inconclusive endomyocardial biopsies, performed due to detection of focal septal scarring on cardiac magnetic resonance imaging, an 18F-FDG-PET-CT, performed upon admission to our clinic, showed distinct hypermetabolic lesions indicative of active inflammation in various organs and raised suspicion of systemic sarcoidosis. Eventually, histopathological evidence of non-caseating granulomas in affected lymph nodes, extracted by bronchoscopy, confirmed the diagnosis of systemic sarcoidosis after reasonable exclusion of other granulomatous diseases. Immediate initiation of long-term immunosuppressive therapy led to almost complete remission, as monitored by consequential 18F-FDG-PET-CT scans.
Discussion
Unexplained complex conduction abnormalities in young patients may be a sign of sarcoidosis, even in isolation of more prevalent symptoms. Correct interpretation and prompt initiation of a structured interdisciplinary diagnostic workup, including 18F-FDG-PET-CT as the imaging modality of choice, are essential to initiate specific treatment and obviate the major risk of mortality resulting from cardiac sarcoidosis.
Publisher
Oxford University Press (OUP)
Subject
Cardiology and Cardiovascular Medicine
Reference29 articles.
1. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous disorders;Hunninghake;Sarcoidosis Vasc Diffuse Lung Dis,1999
2. Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan;Iwai;Acta Pathol Jpn,1993
3. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis;Silverman;Circulation,1978
4. Cardiac sarcoidosis;Birnie;J Am Coll Cardiol,2016
5. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis;Birnie;Heart Rhythm,2014