Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

Trends in Mortality in Patients with Systemic Sclerosis Over 40 Years: A Systematic Review and Meta-Analysis of Case-Control and Cohort Studies.

Elhai1,  Muriel, Meune2,  Christophe, Avouac1,  Jerome, Kahan1,  Andre, Allanore1,  Yannick

Rheumatology A, Paris Descartes University, Cochin Hospital, APHP, Paris, France, Paris, France
Paris Descartes University, Cardiology department, Cochin Hospital, Paris, France


Among the many different immune-mediated rheumatic diseases, Systemic Sclerosis (SSc) stands out as a severely incapacitating and life-threatening disease, the pathogenesis of which is largely unknown and for which therapeutic options are few and insufficient. Nevertheless, a recent cohort study has suggested a decrease in mortality with 10-year survival rates of 60% in the subgroup of patients included before 1985 as compared to 77% in the subgroup included later one. 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.


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 or case-control studies reporting SSc mortality risk were included. Articles had to report enough data to compute a standardized mortality ratio (SMR). Eligibility of references retrieved by the search was assessed independently by two of the authors and disagreements resolved at each step. We then calculated pooled SMRs of SSc mortality and determined their evolution with time using meta-regression analysis. We also conducted adjusted meta-regression analyses, for the methodology of the studies and for relevant covariates (i.e. age, gender and cutaneous form), respectively.


Among a total of 721 identified references, 637 were excluded on the basis of their title or abstract resulting in 84 articles examined for full text. There were finally 9 independent studies in which SMR was available. They were analyzed corresponding to a total of 2691 patients: mean age: 50.1 years, 2230 (83%) were women, 713/2691 (26%) had diffuse SSc. Mid-cohort year ranged from 1977 to 1995 (<1980: two studies; 1980–90: five studies; >1990: two studies). 732 deaths occurred during a mean follow-up of 7.3 years. The overall pooled SMR was 3.53 [95% CI: 3.03–4.11]. Among 732 deaths, 389/612 deaths (64%) were considered as related or possibly related to SSc whereas 223/612 deaths (36%) were defined as not related to SSc. Cardiac deaths were the most frequent causes of deaths (29%) followed by lung involvement (23%). All adjusted meta-regression analyses did not show any significant change in SMR over time (p=0.523). Exclusion of the studies before 1980 revealed a trend for a decrease in SMR but not significant (p=0.112).


Our results confirmed that SSc is a devastating condition associated with a high risk of mortality reflected by a pooled SMR of 3.5. Despite some data suggesting a decrease in the mortality risk in SSc, SMR has not significantly changed over the last40 years. Further studies are needed to assess the effect of recent available therapies on mortality in SSc.

To cite this abstract, please use the following information:
Elhai, Muriel, Meune, Christophe, Avouac, Jerome, Kahan, Andre, Allanore, Yannick; Trends in Mortality in Patients with Systemic Sclerosis Over 40 Years: A Systematic Review and Meta-Analysis of Case-Control and Cohort Studies. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1456

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