In this population-based study, the overall incidence of SSc was 2.43 per 100,000 (95% CI, 1.83-3.02) and was stable over time. We confirmed the hypothesis that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD. The overall incidence of SSc was higher using the broad criteria (LeRoy et al22) than the narrow criteria (ACR),2 which may have underestimated the actual incidence of SSc. When compared with the literature, incidence and prevalence were similar to the findings of most recent studies in the United States4,6 but were higher than that reported previously in the same population from Minnesota.31,32 The apparent increase in incidence is likely due to a better definition of the disease with the establishment of ACR criteria in 1980. As shown in Table 3, the incidence remained stable over time after these criteria were established. The point prevalence on December 31, 2010, was 39.92 per 100,000 (95% CI, 27.87-51.96), which is similar to studies in the United States using the same ACR criteria.4,6 Although the prevalence of SSc was considered to be higher in the United States than in Japan and Europe,4 the results of this study are close to those of a study in a district of northern Italy,33 challenging the concept of a west-east gradient. The probability of survival was higher than reported previously,6‐9 but similar to a Canadian study in which the overall 5-year and 10-year survival rates were 90% and 82%, respectively,34 emphasizing a trend toward an apparent better outcome of SSc, possibly explained in part by the use of broader criteria. Among the visceral complications, ILD, PAH, CHF, and CKD occurred most frequently. When adjusted for age, the risk of death was not only influenced by the presence of PAH or CKD, but also by ILD itself. Although ILD is now considered a major cause of death in SSc,8,9 the review of the causes of death in the current study suggests that ILD is more a contributing factor than an immediate cause of death in these patients. In this study, we found that PAH was the principal cause of poor outcome among those with SSc. The lengthy time span of our study may be one reason why PAH was a predominant cause of death. Of the SSc-related fatalities from the large EUSTAR database, 35% were attributed to ILD, 26% to PAH, and 26% to cardiac causes.9 Proteinuria, PAH by echocardiography, and reduced FVC were among the independent risk factors for mortality.9 The difference in our study can be explained by a different design (population- vs referral center-based), and a different time frame (the EUSTAR database was initiated in 2004). Nevertheless, both studies emphasize the importance of ILD, PAH, and CKD. Cardiac causes certainly play a role as well, but could not be further established in our study because of the limited number of patients.