Risk of Sudden Death in a General Unbiased Epidemiological Cohort of Sarcoidosis

Background Sarcoidosis is an inflammatory, noncaseating, granulomatous disorder of unknown cause that can affect any body system and is associated with cardiovascular disease including sudden cardiac death (SCD). Cardiac involvement in sarcoidosis is associated with higher risk of SCD, but the SCD risk in the general sarcoidosis population is unknown. We aimed to determine the risk of SCD in people with sarcoidosis versus the matched general population. Methods and Results A population-based cohort of sarcoidosis and age- and sex-matched comparators from January 1, 1976 to December 31, 2013 was used; presence of other comorbidities in the comparator group was not an exclusion criterion. Mortality, including time, place, and cause of death were measured and manually adjudicated for SCD events. Incidence rates are reported per 100 000 person-years, and Cox models were used for group comparisons. Of the 345 incident cases of sarcoidosis (171 men; 50%) there were 58 reported deaths; 10 were definite/probable SCD versus 57 all-cause and 9 SCDs in comparators. Median follow-up was 12.9 years (interquartile range, 6.0-23.4 years) . Incidence rate of SCD in sarcoidosis was 192 (95% CI, 92-352) versus 155 (95% CI, 71-294) in comparators (hazard ratio [HR], 1.28 (95% CI, 0.52-3.17). Nocturnal deaths were more frequent in sarcoidosis 57 (95% CI, 12-168) versus 17 (95% CI, 0.4-95) (HR, 3.76 [95% CI, 0.39-36.47]). No significant differences were detected between the groups by sex, age, calendar year of diagnosis, or disease duration. Conclusions In a population-based cohort of patients with sarcoidosis, the risk for SCD compared with matched comparators was not increased. There were more nocturnal deaths among patients with sarcoidosis, yet this was statistically insignificant.