Mortality in sarcoidosis. A changing pattern of the causes of death

Abstract

Forty-one deaths occurred among 1,090 patients with the clinical diagnosis of sarcoidosis between 1960 and 1977. Thirteen patients died of causes unrelated to sarcoidosis, whereas death was related to sarcoidosis in 28 patients (68%). The most prevalent lesion leading to death was advanced pulmonary involvement (22/28 patients or 78%) with various late complications: cardiorespiratory failure, gram-negative pneumonias, fungal infections. The average clinical course among these 22 patients was 10 years from the onset of the disease. The average age at death was 39 years. Patients who died of central nervous system and cardiac sarcoidosis were younger, and their clinical course was shorter. Subclinical sarcoidosis does not seem to affect life span. Autopsy in 25 patients showed systemic sarcoid lesions in all, including asymptomatic patients and those presenting with clinical manifestations limited to the CNS and the heart. The discrepancy implies difficulty in making the diagnosis, and suggests the need of intensive diagnostic work-up. Improved medical therapy, particularly the control of infections and management of cardiorespiratory failure, has resulted in a protracted clinical course and a changing pattern of the causes of death in sarcoidosis.