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.
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 werc calculated by means of age-and sex-specific mortality rates of the general Danish population.
Result Crude mortality rates were 6.5 and 3.9%/yr. respectively, in men and women.
The standardized mortality ratio (SMR) was 2.9 (95% Cl 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% Cl 3.5 5.7) and patients under the age of 35 yr (SMR 13. 95% Cl 2.7 37). (...)
Mots-clés Pascal : Sclérodermie, Homme, Symptomatologie, Epidémiologie, Mortalité, Etiologie, Danois, Peau pathologie, Tissu conjonctif pathologie, Maladie système, Maladie autoimmune, Immunopathologie
Mots-clés Pascal anglais : Scleroderma, Human, Symptomatology, Epidemiology, Mortality, Etiology, Danish, Skin disease, Connective tissue disease, Systemic disease, Autoimmune disease, Immunopathology
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 98-0369816
Code Inist : 002B07. Création : 25/01/1999.