Christiane Wiefels et al. Olabimpe Lamai Riina Kandolin David Birnie … Eugene Leung Claudio Tinoco Mesquita Rob Beanlands
Introduction
Systemic sarcoidosis (SS) was first described more than a century ago by the Norwegian dermatologist Caesar Boeck. He correlated skin nodules with epithelioid cells with large nuclei and giant cells as “multiple benign sarcoid of the skin”, for their resemblance with sarcoma. Today, the cause of the disease remains unknown, but evidence points toward immunological response to an unidentified antigenic trigger in individuals with genetic susceptibility. The estimation of the prevalence of SS in the population varies in the literature also depending on the type of study: epidemiologic, autopsy or imaging.
Some previous data showed prevalence as high as 100-330 cases per 100,000 inhabitants. , Environmental factors have been attributed to those differences, including sex, age and ethnicity, showing a predisposition for environmental and genetic factors. The reported incidence is similar across sex in North American population but greater in females in Scandinavian and Japanese populations, , but more likely to be chronic and fatal in black Americans. The disease usually develops before the age of 50 years, with a peak incidence at 20 to 39 years old, , and is very uncommon under the age or 15 or older than 70. Symptomatic cardiac sarcoidosis (CS) has been reported in 2-5% of the patients with the systemic form. – However, with the advance of new cardiac imaging techniques, cases of asymptomatic (clinically silent) cardiac involvement have been diagnosed. The disease has been reported in at least 20% of the autopsies and imaging report in the United States to as much as 50% in Japan. ,
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