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Sarcoidosis is a multi-system disease with a wide range of phenotypes. Pulmonary involvement is the most frequently identified target for sarcoidosis and is responsible for the majority of deaths. Cardiac sarcoid is less commonly identified, may be occult, is significantly influenced by race, and can portend an unpredictable and sometimes fatal outcome. Sarcoidosis remains an enigmatic disease spectrum of unknown aetiology, frequently difficult to diagnose and with a variable disease course. This article summarises current views on the diagnosis and management of cardiopulmonary involvement. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a multisystem inflammatory disease, the aetiology of which has still to be resolved. The proposed mechanism is that a susceptible genotype is exposed to one or more potential antigens. A sustained inflammatory response follows, which ultimately results in pathognomonic granuloma formation. Various clinical phenotypes exist with specific genetic associations influencing disease susceptibility, protection, and clinical progression. Occupational and environmental factors, including microbial elements, may then effect the development of this disease. Sarcoidosis is a heterogeneous disease, showing geographic and racial variation in clinical presentation. It demonstrates a familial tendency and clear genotype associations. Additionally, it appears to cluster within closely associated populations (eg, work colleagues) and appears to be related to selected occupations and environmental exposures. Frequently occult, but occasionally fatal, this disease has a very variable prognosis. It is also unusual in having no specific biomarker. The epidemiology and multiple factors that appear to influence the aetiology of sarcoidosis illustrate why this disease state is frequently described as a clinical enigma. [ABSTRACT FROM AUTHOR]

Sarcoidosis remains one of the most enigmatic diseases; often present without symptoms and yet potentially able to affect any organ system with serious consequences. Heart involvement portends probably the worst scenario with patients experiencing symptoms from mechanical dysfunction and the risk of sudden death from rhythm disturbance, both in the form of ventricular tachycardias or atrioventricular heart block. The fascinating aspect of this disease is that, to this day, there is no consensus on the aetiology, no ‘biomarker’ to diagnose the disease and no significant advance in therapy from the long-standing use of steroids. Furthermore, the disease demonstrates different phenotypes in different races, has familial tendency, ‘clusters’ among close associates, and has been linked to numerous environmental (organic and inorganic) and infectious agents. One hundred years after sarcoidosis was first described, this disease spectrum remains one of the most mysterious of human diseases. [ABSTRACT FROM AUTHOR]

Abstract: Cardiac sarcoidosis is an underdiagnosed disease that may be present in as many as 25% of patients with systemic sarcoidosis. Although most commonly recognized in patients with other manifestations of sarcoidosis, it may occur in isolation and its presence is often not appreciated. Cardiac sarcoidosis may present as asymptomatic left ventricular dysfunction, congestive heart failure, atrioventricular block, atrial or ventricular arrhythmia and sudden death. Although untested in clinical trials, early use of high-dose steroid therapy may halt or reverse cardiac damage. This article reviews the clinical manifestations, diagnosis and treatment of sarcoidosis, with an emphasis on new imaging techniques and therapies. [Copyright &y& Elsevier]

To this day the aetiology of sarcoidosis continues to elude definition. Partially as a consequence of this, little in the way of new therapies has evolved. The enigma of this condition is that, unusually for a disease with the potential for devastating consequences, many patients show spontaneous resolution and recover. Cardiac involvement can affect individuals of any age, gender or race and has a predilection for the conduction system of the heart. Heart involvement can also cause a dilated cardiomyopathy with consequent progressive heart failure. The most common presentation of this systemic disease is with pulmonary infiltration, but many cases will be asymptomatic and are detected on routine chest radiography revealing lymphadenopathy. Current advances lie in the newer methods of imaging and diagnosing this unusual heart disease. This review describes the pathology and diagnosis of this condition and the newer imaging techniques that have developed for determining cardiac involvement. [ABSTRACT FROM AUTHOR]