Cardiac sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology that is histologically characterised by non-caseating granulomas. Hilar lymph nodes, lung, eyes and skin are the organs most commonly affected, although the heart, liver, spleen and bones may also be involved. Cardiac involvement is relatively rare and is associated with a poor prognosis. In addition to clinically manifest heart failure, the most common clinical manifestations are arrhythmias and sudden cardiac death. The absence of clinical symptoms does not rule out cardiac sarcoidosis, because most cases are detected as late as at necropsy. The diagnosis is based on a series of diagnostic approaches, such as electrocardiography, echocardiography, radionuclide imaging, magnetic resonance imaging, coronary angiography, endomyocardial biopsy and electrophysiological testing. However, the test results often do not allow the diagnosis to be made, and response to therapy is the only confirmation of the anticipated diagnosis. Various therapeutic modalities include treatment with immunosuppressive agents and specific cardiac medication, and implantation of a pacemaker or an implantable cardioverter defibrillator. If these methods fail to succeed, heart transplantation remains the therapy of choice.