Background Systemic sclerosis (SSc) is an uncommon autoimmune multisystem disease associated with reduced life expectancy compared with the general population.1 In order to prolong survival of this patient population, clear information on the most important death-related conditions is undoubtedly necessary. No mortality data in SSc, however, are available from Latin America, as well as few large series studies have looked at the mortality profile in SSc.
Objectives We aimed to describe the causes of death in SSc occurred in the state of Rio de Janeiro, Brazil, from 2006–2015, and also to compare the data gathered with the general population mortality.
Methods All death certificates issued in the state of Rio de Janeiro, Brazil, from 2006–2015 were screened for the code attributed to SSc according to the tenth revision of the International Classification of Diseases (ICD-10), either as an underlying (UD) cause of death (also referred to as basic cause of death) or a non-underlying (non-UD) cause. In addition to compiling the causes of death in both settings, we calculated the non-adjusted as well as the age bracket-adjusted (age at death <50 years and ≥50 years) mortality ratio against the general population for each cause of death when SSc was listed as a non-UD cause.
Results Of 1.294.491 fatalities recorded over the study period, ICD-10 code for SSc was listed on 374 (0.02%) death certificates, being a basic cause of death on 223 occasions and a non-UD cause on 151 occasions. The overall mean (SD) age at death in SSc was 58.7 (15.6) years, with men (n=56) having an earlier mean age at death than women (n=318) [53.5 vs 59.6 years, respectively; p=0.004]. For SSc as a basic cause of death, the main non-UD causes were respiratory system diseases (61.4%), in particular pneumonia, followed by septicemia (37.6%), diseases of the circulatory system (34.9%), and renal failure (9.4%). There were no significant differences between the genders for each cause of death. The mean age at death was significantly lower among men vs women for diseases of the respiratory system, respiratory failure, certain infectious and parasitic diseases, and septicemia. For SSc as a non-UD cause of death, the major conditions leading to death were circulatory system diseases (39%), in particular pulmonary arterial hypertension (PAH; 13.2%), followed by certain infections and parasitic diseases (11.9%), malignant neoplasms (10.5%) and diseases of the digestive system (9.9%). Compared with the overall population, patients with SSc had an excess of death (odds ratio [OR] >1) due to PAH (OR 138.94), septicemia (OR 1.92), gastrointestinal hemorrhage (OR 2.40), other systemic connective tissue diseases (OR 24.78) and pulmonary fibrosis (OR 11.05), as well as due to heart failure (OR 6.40) for deaths occurred before age 50.
Conclusions We have shown large data on the mortality profile of patients with SSc, the first from Latin America. Of note, infections and cardiorespiratory diseases had a strong impact on mortality, as evidenced by previous publications.1 Taking all into account, these data support an increased vigilance for infections, as well as the need to implement effective measures to control modifiable cardiovascular risk factors, including screening for PAH.
Rubio-Rivas M, et al. Semin Arthritis Rheum 2014; 44:208–219.
Disclosure of Interest None declared