Epidemiology of Sarcoidosis: Recent Advances and Future Prospects

Abstract

Sarcoidosis is by definition a disease of "unknown causes," but recent epidemiologic advances suggest that the long-standing definition of sarcoidosis may soon need to be amended. The recently completed ACCESS (A Case-Control Etiologic Study of Sarcoidosis) study was not able to definitively identify the "cause" of sarcoidosis, but yielded important findings regarding familial and environmental risks that have advanced our understanding of this disease. The HLA-DRB1 associations reported in ACCESS along with the results of two recently completed genome scans of sarcoidosis in German Caucasians and African-Americans, respectively, have further defined the genetics of sarcoidosis. These studies suggest genetic heterogeneity of sarcoidosis risk between Caucasians and African-Americans and multiple susceptibility genes that interact together and with environmental factors in the disease pathogenesis. Genes that influence sarcoidosis clinical phenotypes may also be largely separate from sarcoidosis susceptibility genes. Although genetic studies of sarcoidosis in African-American populations are confounded by Caucasian admixture, this same admixture may be useful in identifying sarcoidosis genes linked with African ancestry. Case-only methods may be useful in identifying recent acute exposures linked to disease, genetic variants of risk, and gene-environment interactions. In summary, the epidemiology of sarcoidosis has a promising future that should eventually provide the answers to the etiologic origins of this complex disease.

Global burden of chronic pulmonary aspergillosis complicating sarcoidosis

“…The Chinese, Malay and Indian incidence rates in Singapore were 0.56, 1.3 and 4.57 per 100,000 [25] and, in London, the rates of Caucasians, Indians and black people were 1.5, 16.8 and 19.8 per 100,000 [26]. In the USA, the male Caucasian rate was 9.6, whereas it was 29.8 in black people [24]. NUNES et al [40] proposed a global annual incidence of sarcoidosis of 17.8 per 100,000.…”

“…Proportions per 100,000 population ranged from 0.1 to 64 [14][15][16]. Available individual country prevalences were used for the UK [17], Israel [18], Iceland [19], Spain (pulmonary sarcoidosis only) [20], Greece [21], Switzerland [22], Japan [23] and the USA [24]. Extrapolations were made as follows: China and Vietnam were assumed to be similar to Singapore Chinese [25]; India, Pakistan and Bangladesh, to Singapore Indians [25]; Philippines, Thailand and Indonesia, to Singapore Malaysians [25]; Congo, Nigeria and Ethiopia, to UK black people [26]; Egypt and Iran, to Turkey [27]; and Russia, to Finland [23].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The etiology of sarcoid is unknown, but it is presumed to be related to an immune response toward an unidentified antigen. Sarcoid affects African-Americans more commonly than Caucasians with the incidence in North America estimated to be and 350 to 800 per 1 million African Americans and 30 to 100 per 1 million Caucasians of the population [178]. Females are affected more often than males [178].…”

Neurosarcoidosis: An Illustrative Case and Review of Perioperative Considerations

“…The overall disease prevalence in the United States is estimated at 40 per 100,000 with an annual incidence of 0.035% to 0.080% among African Americans and 0.003% to 0.01% in whites [1]. As for sarcoidosis confined solely to the nervous system, the annual incidence is estimated at less than 0.2 per 100,000 among whites [6].…”

“…The incidence of sarcoidosis in North America is estimated at 3 -10 per 100,000 among Caucasians and 35 -80 per 100,000 among African Americans [1]. Neurosarcoidosis is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in patients with systemic sarcoidosis.…”