Distinct mortality profile in systemic sclerosis: a death certificate study in Rio de Janeiro, Brazil (2006–2015) using a multiple causes of death analysis

• Published in: Clinical rheumatology Vol. 38; no. 1; pp. 189 - 194
• Main Authors: de Rezende, Rodrigo Poubel Vieira, Gismondi, Ronaldo Altenburg, Maleh, Haim Cesar, de Miranda Coelho, Elisa Mendes, Vieira, Carol Sartori, Rosa, Maria Luiza Garcia, Mocarzel, Luis Otavio
• Format: Journal Article
• Language: English
• Published: London Springer London 01.01.2019; Springer Nature B.V
• Subjects: Adolescent; Adult; Age; Age Distribution; Aged; Aged, 80 and over; Brazil - epidemiology; Brief Report; Cardiovascular Diseases - complications; Cause of Death; Circulatory system; Connective tissue diseases; Coronary artery disease; Death; Death Certificates; Female; Fibrosis; Heart diseases; Hemorrhage; Humans; Infection - complications; Lung diseases; Male; Medicine; Medicine & Public Health; Middle Aged; Mortality; Pulmonary hypertension; Registries; Respiratory diseases; Respiratory Tract Diseases - complications; Retrospective Studies; Rheumatology; Scleroderma; Scleroderma, Systemic - mortality; Septicemia; Sex Distribution; Systemic sclerosis; Young Adult; Brazil; Rio de Janeiro Brazil; Infections; Causes of death; Mortality profile; Mortality; Systemic sclerosis
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-017-3951-8

The objective of this study was to assess the mortality profile related to SSc in the state of Rio de Janeiro, Brazil. We retrospectively examined all registered deaths in the region (2006–2015 period) in which the diagnosis of SSc was mentioned on any line of the death certificates (underlying cause of death [UCD], n  = 223; non-UCD, n  = 151). Besides the analysis of gender, age, and the causes of death, we also compared the mortality from UCDs between individuals whose death causes included SSc (cases) and those whose death causes did not include SSc (deceased controls). For the latter comparison, we used the mortality odds ratio to approximate the cause-specific standardized mortality ratio. We identified 1495 death causes among the 374 SSc cases. The mean age at death of the SSc cases (85% women) was significantly lower than that of the controls ( n  = 1,294,117) (58.7 vs. 65.5 years, respectively). The main death causes were circulatory system diseases, infections, and respiratory diseases (36%, 34%, and 21% of SSc cases, respectively). Compared to the deceased controls, there were proportionally more deaths among the SSc cases from pulmonary arterial hypertension, lung fibrosis, septicemia, gastrointestinal hemorrhage, other systemic connective tissue diseases, and heart failure (for death age < 50 years). We confirmed the high burden of cardiovascular, respiratory, and infectious causes in this predominantly non-Caucasian sample of SSc patients. Of interest, the percentage of infection-related deaths in our report was about three times higher than that in SSc studies with predominantly Caucasian populations.