Christophe Meune

OBJECTIVE: SSc is known as the most severe connective tissue disorder, and to be associated with a high mortality risk. Some improvements in therapy for SSc have been achieved in recent years and some preliminary data have suggested an improvement in patient survival. Thus, we set out to determine whether mortality rate in SSc patients has decreased over the past 40 years through a meta-analysis of cohort studies. METHODS: We performed a systematic review and a meta-analysis of literature in MEDLINE and Embase databases from January 1960 to June 2010. All cohort studies reporting on SSc mortality were analysed. We then calculated pooled standardized mortality ratios (SMRs) of SSc mortality and calculated their changes over time using meta-regression analysis. RESULTS: Nine studies were included, corresponding to a total of 2691 SSc patients. The pooled SMR was 3.53 [95% CI 3.03, 4.11, P < 0.0001; I(2 )= 93%, P(het) = 0.001]. Mid-cohort year ranged from 1977 to 1995 (before 1980: two studies; 1980-90: five studies; and after 1990: two studies): adjusted meta-regression analysis did not show significant change in SMR over time (P = 0.523). Among 732 deaths, heart involvement was the most frequent cause of deaths (29%) followed by lung involvement. CONCLUSION: Our results confirm that SSc is a devastating condition as reflected by a pooled SMR of 3.5. Additionally, SMR has not significantly changed over the past 40 years. Further studies are needed to assess the effect of the most recent available therapies on mortality in SSc.

OBJECTIVES: RA is known to be associated with a high cardiovascular (CV) risk. Longitudinal data suggest that RA disease course may have become milder over the past decades. Thus, we set out to estimate the magnitude of the overall increase in CV mortality associated with RA and to determine whether it has decreased over the past 50 years. METHODS: We performed a systematic review and a meta-analysis of literature in MEDLINE and EMBASE databases from January 1960 to November 2008. All cohort studies reporting CV mortality risk were included. We then calculated pooled standardized mortality ratios (SMRs) of CV mortality, and determined their evolution with time using meta-regression analysis. RESULTS: Seventeen studies were analysed, corresponding to a total of 91 916 patients. The overall pooled SMR was 1.6 (95% CI 1.5, 1.8; I(2) = 93%; P(het) < 0.0001). Mid-cohort year ranged from 1945 to 1995 (<1980, seven studies; 1980-90, five studies; >1990, five studies). Meta-regression analyses revealed neither any trend in SMR over time (P = 0.784) nor any relation with disease duration at the time of inclusion (P = 0.513). CONCLUSIONS: Our results show that RA is associated with a 60% increase in risk of CV death compared with general population. Despite changes in RA course over the past decades, SMR for CV death has not changed. This suggests that targeting a reduction in CV mortality should still be considered as a major issue in RA.

To the Editor: Lamin A/C gene mutations are associated with various disorders,1,2 including cardiac abnormalities characterized by atrial fibrillation, conduction-system disturbances, sudden death,...