Objectives To evaluate extrapulmonary activity and correlate baseline features at diagnosis with survival in a large cohort of patients with sarcoidosis.
Methods We studied 170 patients consecutively diagnosed with sarcoidosis in the last 10 years. The diagnosis of sarcoidosis was based on clinical criteria and imaging studies, together with the histopathological demonstration of non-necrotizing granulomas, and the exclusion of other granulomatous diseases, especially infections (tuberculosis). Extrapulmonary involvement was defined according to the criteria defined by Judson et al, 2011.
Results There were 111 (65%) women and 59 (35%) men, with a mean age at diagnosis of 47.49 years (range 17-92). The main clinical manifestations present at diagnosis were respiratory symptoms in 112 (66%) patients, erythema nodosum in 35 (21%), articular involvement in 35 (21%) and fever in 34 (20%) patients; incidental imaging findings in asymptomatic patients was found in 12% of cases. After a mean follow-up of 107.4 months, 21 (12%) patients died. The main causes of death were infections in 4 patients, cardiovascular disease in 2, pulmonary sarcoidosis in 1, neoplasia in 1 and other causes in 2 patients; in the remaining 11 patients, the cause of death was unknown or unclassifiable. Survivors had a lower mean age (44.82 yrs. vs. 65.95 yrs. p<0.001) and a statistical trend to higher mean levels of serum angiotensin-converting enzyme (ACE) (105.78 U/L vs. 64.58 U/L, p=0.16). The mortality rate was higher in patients born in Spain (16% vs. 0% in those born outside Spain, p=0.008). Extrapulmonary involvement was unrelated to death (mortality rate of 11% vs. 14% in patients with pulmonary involvement). Multivariate analysis identified mean age at diagnosis (p<0.001) as an independent risk factor associated with death.
Conclusions Extrapulmonary involvement in sarcoidosis is not associated with mortality. An older age at diagnosis was the principal risk factor associated with death, while patients born outside Spain had a lower mortality rate, and mean ACE levels at diagnosis were inversely associated with survival. The very-low rate of deaths caused directly by sarcoidosis might explain our results.
Disclosure of Interest None declared
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