The British Journal of Rheumatology, Vol 37, 750-755, Copyright © 1998 by British Society for Rheumatology
S Jacobsen, P Halberg and S Ullman
OBJECTIVE: To determine survival, mortality and causes of death in Danish
patients with systemic sclerosis (scleroderma), and to analyse how these
parameters are influenced by demographic variables and the extent of skin
involvement. METHODS: A cohort of 344 patients with incident systemic
sclerosis (SSc) after 1 January 1960 was retrospectively identified,
representing 3716 patient-years of follow- up. The vital status at the end
of the study ultimo 1996 was established by reviewing the clinical charts
and by contacting the Danish Central Person Register. The causes of death
were based on information obtained from the hospital charts and the autopsy
reports (80%) and death certificates (20%). The extent of skin sclerosis
was described by means of a two-subset model (limited and diffuse
involvement) and a three-subset model (digital, extremity and truncal
involvement) in all patients. Expected numbers of deaths were calculated by
means of age- and sex-specific mortality rates of the general Danish
population. RESULTS: Crude mortality rates were 6.5 and 3.9%/yr,
respectively, in men and women. The standardized mortality ratio (SMR) was
2.9 (95% CI 2.5-3.4) and was not significantly influenced by sex or age at
disease onset. Mortality rates were significantly increased in patients
with diffuse skin sclerosis (SMR 4.5, 95% CI 3.5-5.7) and patients under
the age of 35 yr (SMR 13, 95% CI 2.7-37). Of the 160 patients who died
during the study, 41 deaths (26%) were SSc related, accounting for
one-third of the excess mortality. CONCLUSION: We found a significantly
increased mortality in patients with SSc, particularly in the subset of
patients with diffuse skin sclerosis and in young patients. The excess
mortality was due to an increase in both the SSc-related mortality and the
unrelated mortality. However, all other subsets also had a significantly
increased risk of death, mainly due to an increased risk of death due to
causes unrelated to SSc, cancer among others. The fact that the unrelated
mortality in SSc was found to be 2-fold increased suggests that the excess
mortality that was classified as unrelated should more appropriately be
termed indirectly SSc related.
ORIGINAL PAPERS
Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)
Department of Rheumatology, Copenhagen University Hospital at Hvidovre, Denmark.
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