Systemic sclerosisDeterminants of Morbidity and Mortality of Systemic Sclerosis in Canada
Section snippets
Study Design
This was a cohort study. Demographic and clinical characteristics of SSc patients were compared between those who survived versus those who died during follow-up in a rheumatology SSc practice between 1994 and 2004.
Data Collection
The database was constructed using the medical records of patients with a diagnosis of SSc who met preliminary American College of Rheumatology criteria (19), or who had CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) (20).
Results
One hundred eighty-five patients were included in the analysis (158 women and 27 men). Thirty-seven percent of the patients had dcSSc and 63% had lcSSc (Table 1). At the time of analysis, the mean follow-up time until death or last visit was 9.1 years (similar between those living and those deceased) (Table 2), and 23% of the total cohort were deceased. Thirty-seven percent were smokers and another 9% had been past smokers. The 5-year survival was 90% (95% for limited and 81% for diffuse) and
Demographic Characteristics
The sex-specific differences observed in our scleroderma cohort were consistent with the published literature, in which men had earlier age of disease onset (3), a greater likelihood of having dcSSc (3, 21), and an earlier average age of death (3, 13). Age at diagnosis was only slightly younger for those with dcSSc compared with lcSSc, and this may reflect a trend for increased early detection of lcSSc, as historically lcSSc has been thought to onset later than dcSSc.
Mortality in SSc
We compared our data to 2
References (48)
Scleroderma epidemiology
Rheum Dis Clin North Am
(2003)Scleroderma renal crisis
Rheum Dis Clin North Am
(2003)- et al.
Prediction of survival in progressive systemic sclerosis by multivariate analysis of clinical features
Am Heart J
(1985) - et al.
Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002
Semin Arthritis Rheum
(2005) - et al.
Mortality in systemic sclerosis: an international meta-analysis of individual patient data
Am J Med
(2005) Connective tissue diseases characterized by fibrosis
Systemic sclerosis. B. Clinical features
Epidemiology of systemic sclerosis
Epidemiologic studies of environmental agents and systemic autoimmune diseases
Environ Health Perspect
(1999)- et al.
Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients
Medicine (Baltimore)
(2002)
Scleroderma—demographics and survival
J Rheumatol
Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population
Arthritis Rheum
Mortality in systemic sclerosis: a comparison with the general population
J Rheumatol
Predictors of survival in systemic sclerosis (scleroderma)
Arthritis Rheum
Epidemiology of systemic sclerosis (scleroderma)
Ann Intern Med
Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors
Ann Intern Med
Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival
Medicine (Baltimore)
A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease
J Rheumatol
Severe organ involvement in systemic sclerosis with diffuse scleroderma
Arthritis Rheum
Cigarette smoking as a significant risk factor for digital vascular disease in patients with systemic sclerosis
Arthritis Rheum
Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis
Arthritis Rheum
Preliminary criteria for the classification of systemic sclerosis (scleroderma)
Arthritis Rheum
Scleroderma (systemic sclerosis): classification, subsets and pathogenesis
J Rheumatol
Cutaneous and serologic subsets of systemic sclerosis
J Rheumatol
Cited by (175)
Accelerated Atherosclerosis and Management of Cardiovascular Risk in Autoimmune Rheumatic Diseases: An Updated Review
2023, Current Problems in CardiologyPreventative Care in Scleroderma: What Is the Best Approach to Vaccination?
2023, Rheumatic Disease Clinics of North America
This research was funded in part by the University of Western Ontario Summer Research Training Program (SRTP) grant.