Scleroderma and survival

Article Abstract:

Scleroderma, also called systemic sclerosis, is a condition that affects the skin and connective tissue. It is a rare disease and is fatal when more than one of the major body organs (lungs, heart, kidneys) are affected. Mortality from scleroderma is higher than from other connective tissue diseases such as systemic lupus erythematosus. Several studies have reported that mortality from scleroderma is greater among men than women and increases with age. However, it should be noted that mortality for the general population follows the same pattern, thereby making it difficult to identify trends in mortality from scleroderma. Factors associated with a poor chance of survival in patients with scleroderma include alcohol consumption and cigarette smoking (for men), and blacks have been reported to have lower survival rates than whites one year after diagnosis. The extent of skin sclerosis has been used as a measure of the severity of the disease and to estimate the outcome. Two recent studies have reported a relationship between the extent of the skin sclerosis and survival. Ten-year survival rates were reported to be less than 25 percent for patients with internal organ damage, and were higher for those with disease confined to the wrists and ankles. In a study that included 646 patients with differing degrees of sclerosis, 24 patients had kidney disease and 75 percent of them died within one year, while the rest died within six years. For those with disease affecting the heart, the survival rate was 50 percent by three years, and all died within nine years. Sclerosis of the lungs was associated with a 10-year survival rate of 50 percent, and the survival of patients with no major organ sclerosis was 70 percent at 10 years after diagnosis. To date, there is no treatment for sclerosis that improves the outcome or survival. The extent of the sclerosis is still the best indicator of survival. (Consumer Summary produced by Reliance Medical Information, Inc.)

Connective tissue diseases

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Epidemiology of scleroderma

Article Abstract:

Scleroderma is a rare disease, and most epidemiological information has been obtained from patients treated in health clinics with defined focuses. The death rate from the disease is low at young ages and increases slowly with advancing age. The ratio of women to men with scleroderma decreases with age, from 7:1 in early adulthood to about 3:1 in the fifth decade of life. The disease varies in severity, probably occurring in several subtypes, and the true occurrence is unclear; at about 290 cases per million population, it is probably underestimated. Distribution of the disease is apparently unrelated to urban or rural life, but may be somewhat higher in US blacks, and varies among countries. It appears to be more common in the South Atlantic and East South Central areas of the US. An increased risk of miscarriage may be associated with scleroderma. Due to the rarity of the disease, familial tendencies are uncommon. Compared with other rheumatic diseases, the occurrence of scleroderma is only weakly linked to particular HLA antigens (human leukocyte antigen). Environmental agents have been implicated in the development of scleroderma, including silica (usually studied in miners), varied organic solvents, toxic oil (rapeseed oil contaminated with aniline), drugs, and breast augmentation with silicone implants. Breast cancer may possibly develop in some women at the same time as scleroderma. Patients with the disease appear to have a higher risk of lung cancer, independent of smoking but related to lung fibrosis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Demographic aspects, Epidemiological research, Cross sectional studies

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Mortality from scleroderma in England and Wales 1968-1985

Article Abstract:

Scleroderma is a disease that affects the skin and certain internal organs; the skin texture becomes tough and taut, and fluid is retained underneath. It is a rare disease with a high rate of fatality. In order to estimate the incidence of this disease in England and Wales, death certificates between the years 1968 and 1985 were reviewed. The results of this study showed that on a yearly basis, scleroderma was responsible for 1 out of every million deaths for men and 4 out of every million deaths for women. The largest number of deaths caused by scleroderma occurred in the age group 65 to 74 years. Between 1968 and 1985, the number of deaths caused by scleroderma increased at a rate of 3 percent each year. This indicates an increase in the number of deaths by approximately 50 percent over the entire 17-year study period. These results are similar to those previously published in a study of scleroderma mortality conducted in the United States. The estimated mortality from scleroderma in the United States is 1.5 and 3.5 per million deaths per year for men and women, respectively. It is estimated that 60 percent of the women and more than 60 percent of the men with scleroderma die from their disease. The results of this study show that mortality from scleroderma in England and Wales is similar to that in the United States, and that the increase in incidence of the disease is likely to be similar as well. (Consumer Summary produced by Reliance Medical Information, Inc.)

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