Clinical research study
Long-Term Prognosis and Causes of Death in Systemic Lupus Erythematosus

https://doi.org/10.1016/j.amjmed.2005.11.034Get rights and content

Abstract

Purpose

We studied survival rate, prognostic factors, and causes of death in patients with systemic lupus erythematosus (SLE), particularly focusing on the influence of disease severity.

Patients and Methods

A cohort of 207 consecutive Italian patients with SLE were prospectively studied. All prominent clinical and serologic parameters were evaluated and considered as prognostic risk factors. Causes of death were defined on the basis of clinical data and, when available, postmortem examination. Survival was calculated from the time of diagnosis by Kaplan-Meier method.

Results

A total of 17 of 207 patients died; causes of death were active disease manifestations in 35.3% of cases and complication of the disease or its treatment in 64.7% of cases. The survival rates at 5, 10, and 15 years after the diagnosis were 96%, 93% and 76%, respectively. By multivariate analysis of the risk factors, a predictive model consisting of male gender, positive lupus anticoagulant, and “severe” SLE was identified. The survival curve of the patients with severe disease was similar to that of patients with mild disease until 10 to 15 years from the diagnosis. Thereafter the two curves tended to diverge, showing a clear survival decline in patients with severe disease.

Conclusions

Our study confirms the increase of short- and medium-term survival in patients with SLE, but long-term prognosis remains poor in patients with severe SLE manifestations.

Section snippets

Patients and methods

We recruited 207 consecutive patients with an SLE diagnosis who were seen at the Division of Rheumatology, Padua University, during a 40-year period. A total of 181 patients were female and 26 were male (female:male ratio 7:1). The mean (±standard deviation) age at diagnosis was 32.3 ± 9 years.

All patients satisfied at least four of the American College of Rheumatology (ACR) criteria for the Classification of SLE15 and were managed according to diagnostic and therapeutic guidelines similar to

Results

The main clinical manifestations and laboratory abnormalities of 207 patients are summarized in Table 1. Ninety-two patients (44.4 %) had severe SLE, and 115 patients (55.6 %) had mild SLE. At the time of baseline evaluation, the patients had an average European Consensus Lupus Activity Measure score of 3.32 ± 1.26 (range 1-8), and 21 patients (10%) had damage in at least one of the organ systems considered in the Systemic Lupus International Collaborating Clinic and ACR Damage Index.

Discussion

Our study was performed on a cohort of 207 patients followed up for a long period of time. All of the patients satisfied the ACR criteria15 for SLE classification and were managed according to a protocol similar to that recently developed by the ACR.13

The mortality we observed in this cohort was similar to that reported in the most recent studies in which it varied from 6.8% to 20.2% in SLE cohorts followed up for periods ranging from 8 to 14 years.3, 23, 24, 25 The survival rates at 5, 10, and

Conclusion

Our study confirms the increase of short- and medium-term survival in patients with SLE. The drugs used for the treatment of the severe manifestations of SLE, particularly glomerulonephritis, seem to reduce the overall mortality caused by disease activity during the first 10 to 15 years after the diagnosis, but, unfortunately, they may induce severe side effects leading to a poor long-term prognosis.

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