Cardiovascular disease a hazard despite improved prognosis in patients with systemic lupus erythematosus: results from a Swedish population based study 1964-95.

OBJECTIVE Although short term prognosis has improved in patients with systemic lupus erythematosus (SLE) during the early disease course, less is known about the longterm prognosis. METHODS A cohort of 4737 patients with a diagnostic code of SLE was identified 1964-94 in the Swedish Hospital Discharge Register and followed by linkage to the Cause of Death Register until the end of 1995. Mortality was separately analyzed in 3 different calendar periods (1964-75, 1975-84, 1985-95). The relative risk of death was estimated as standardized mortality ratio (SMR) using the Swedish population as a reference. RESULTS In total 2314 patients were deceased. Mortality was 3-fold increased (SMR = 3.63, 95% CI 3.49, 3.78) and cardiovascular disease (CVD) was the major cause of death. Patients aged 20-39 years at the first discharge had a 16-fold increased risk of death from coronary heart disease (SMR = 15.99, 95% CI 10.4, 23.6). All-cause mortality had decreased since 1975 and the reason for this decrease was entirely due to a decrease in causes attributed to SLE, but not CVD. Patients aged 20-39 years at the first discharge had a pronounced decrease in mortality, with SMR 33.59 (95% CI 24.3, 45.3) before 1975 compared with SMR 14.23 (95% CI 8.70, 22.0) after 1984. CONCLUSION Cardiovascular disease was the major cause of death in patients with SLE and young patients had a pronounced increased risk of death. Even if all-cause mortality had declined during the last 2 decades due to causes attributed to SLE, the risk of cardiovascular death remained unchanged.

[1]  L. Pasquier,et al.  Orphanet Journal of Rare Diseases , 2006 .

[2]  G. Pennello,et al.  Cause-specific mortality in a cohort of patients with diabetes mellitus: a population-based study in Sweden. , 2001, Journal of clinical epidemiology.

[3]  O. Nived,et al.  Incidence studies of systemic lupus erythematosus in Southern Sweden: increasing age, decreasing frequency of renal manifestations and good prognosis. , 2000, The Journal of rheumatology.

[4]  J. Cameron,et al.  The very long-term prognosis and complications of lupus nephritis and its treatment. , 1999, QJM : monthly journal of the Association of Physicians.

[5]  L. Kuller,et al.  Prevalence and risk factors of carotid plaque in women with systemic lupus erythematosus. , 1999, Arthritis and rheumatism.

[6]  A. Voss,et al.  Mortality and causes of death of 513 Danish patients with systemic lupus erythematosus. , 1999, Scandinavian journal of rheumatology.

[7]  J Thumboo,et al.  Trends in the incidence and mortality of systemic lupus erythematosus, 1950-1992. , 1999, Arthritis and rheumatism.

[8]  Lars Klareskog,et al.  Disease activity and risk of lymphoma in patients with rheumatoid arthritis: nested case-control study , 1998, BMJ.

[9]  D. Gladman,et al.  Mortality studies in systemic lupus erythematosus. Results from a single center. III. Improved survival over 24 years. , 1997, The Journal of rheumatology.

[10]  S. Studenski,et al.  Causes of death in systemic lupus erythematosus. Long-term followup of an inception cohort. , 1995, Arthritis and rheumatism.

[11]  D. Gladman,et al.  Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death. , 1995, The Journal of rheumatology.

[12]  E. Bergstralh,et al.  Prognostic determinants in lupus nephritis: a long-term clinicopathologic study , 1995, Lupus.

[13]  O. Johnell,et al.  Cancer risk after hip replacement with metal implants: a population-based cohort study in Sweden. , 1995, Journal of the National Cancer Institute.

[14]  H. Adami,et al.  The epidemiology of inflammatory bowel disease: a large, population-based study in Sweden. , 1991, Gastroenterology.

[15]  K. Steinsson,et al.  Systemic lupus erythematosus in Iceland 1975 through 1984. A nationwide epidemiological study in an unselected population. , 1990, The Journal of rheumatology.

[16]  A. Silman,et al.  Estimating the incidence of systemic lupus erythematosus in a defined population using multiple sources of retrieval. , 1990, British journal of rheumatology.

[17]  H. Adami,et al.  Time trends in incidence rates of first hip fracture in the Uppsala Health Care Region, Sweden, 1965-1983. , 1989, American journal of epidemiology.

[18]  L. Nyström,et al.  A validation of cause of death certification for ischaemic heart disease in two Swedish municipalities. , 1988, Scandinavian journal of primary health care.

[19]  L. Råstam,et al.  The Skaraborg hypertension project. I. The prevalence of hypertension. , 2009, Acta medica Scandinavica.

[20]  J. Fries,et al.  A multicenter study of outcome in systemic lupus erythematosus. II. Causes of death. , 1982, Arthritis and rheumatism.

[21]  P. Allebeck,et al.  Increased mortality among persons with rheumatoid arthritis, but where RA does not appear on death certificate. Eleven-year follow-up of an epidemiological study. , 1981, Scandinavian journal of rheumatology.

[22]  L. Wilhelmsen,et al.  Treatment of hypertension in the community. A preliminary report. , 2009, Acta medica Scandinavica. Supplementum.

[23]  U. de Faire,et al.  A validation of cause-of-death certification in 1,156 deaths. , 2009, Acta medica Scandinavica.

[24]  M. Urowitz,et al.  The bimodal mortality pattern of systemic lupus erythematosus. , 1976, The American journal of medicine.

[25]  Roberto Iotti,et al.  [Causes of death in systemic lupus erythematosus]. , 1970, Prensa medica argentina.

[26]  John C. Bailar,et al.  202. Note: Significance Factors for the Ratio of a Poisson Variable to Its Expectation , 1964 .